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Manifestations and treatment options for eosinophilic leukemia. Chronic myeloproliferative neoplasms Acute myeloid leukemia - symptoms of promyelocytic, monoblastic, myelomonocytic myeloid leukemia

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Chronic myeloproliferative neoplasms (MPN) are a clonal hematopoietic stem cell disorder characterized by proliferation of one or more myeloid lineages (granulocytic, erythroid, megakaryocyte and mast cell).

According to the WHO classification (2008), depending on the predominance of lesions of certain cell lines, the following nosological forms are included in this group.

Myeloproliferative neoplasms:

Chronic myeloid leukemia, BCR-ABL1 positive
- chronic neutrophilic leukemia
- polycythemia vera
- primary myelofibrosis
- essential thrombocythemia
- chronic eosinophilic leukemia, unspecified (CEL NS)
- mastocytosis
- myeloproliferative neoplasm, unclassified (NC)

Myelodysplastic/myeloproliferative neoplasms (MDS/MPN):

Chronic myelomonocytic leukemia
- atypical chronic myeloid leukemia BCR-ABL1 negative
- juvenile myelomonocytic leukemia
- myelodysplastic/myeloproliferative neoplasms, unclassified
conditional form: refractory anemia with ring sideroblasts and thrombocytosis

Myeloid and lymphoid neoplasms associated with eosinophilia and PDGFRA, PDGFRB or FGFR1 abnormalities:

Myeloid and lymphoid neoplasms associated with PDGFRA rearrangement
- myeloid neoplasms associated with PDGFRB rearrangement
- myeloid and lymphoid neoplasms associated with FGFR1 abnormalities

Below are the main clinical, hematological and laboratory data of some of the above nosological forms (H. Bonner, A. J. Erslev, 1994).

Table 1. Basic clinical, hematological and laboratory data of nosological forms

Chronic myelomonocytic leukemia

Chronic myelomonocytic leukemia (CMML) refers to myeloproliferative neoplasms. In 1996, the FAB group proposed to distinguish between two variants of CMML. When the number of leukocytes is less than 13.0x10 9 /l, the MDS-CMML variant is indicated; when leukocytosis is higher than 13.0x10 9 /l, the MPN-CMML variant is indicated. It has been shown that in the absence of significant differences in clinical and hematological signs, survival with the MDS-CMML variant is higher than with the MPN-CMML variant.

Chromosomal abnormalities occur in 20-15% of patients: del 7q, trisomy 8, der/del 12p; but del 5q- is not found in this variant.

In CMML, excessive proliferation of monocytes can cause splenomegaly (in 17% of patients) and hepatomegaly (in 13% of patients); lymphadenopathy and hyperplastic gingivitis are sometimes observed.

According to the WHO classification, the following diagnostic criteria for CMML are distinguished:

Peripheral blood monocytes more than 1.0x10 9 /l,
- less than 20% blasts in the blood or bone marrow,
- absence of a Ph chromosome or BCR/ABL fusion gene,
- dysplasia of one or more myeloid lines;

In the absence or minimal myelodysplasia, the diagnosis of CMML can be made if:

Acquired clonal cytogenetic abnormalities in the bone marrow, or
- in the presence of constant monocytosis during the last three months with the exclusion of other causes of monocytosis.

Diagnosis of CMML-1 - if present
The diagnosis of CMML-2 is the presence of 5-19% blasts in the blood, 10-19% in the bone marrow, or the presence of Auer rods and the presence of blasts less than 20% in the blood or bone marrow.

The diagnosis of CMML-1 or CMML-2 with eosinophilia is made if, in addition to these criteria, the number of eosinophils in the blood is higher than 1.5x10 9 /l.

In this classification of CMML, the number of blasts includes myeloblasts, monoblasts and promonocytes.

Differential diagnosis is carried out with CML and variants M4, M5 acute myeloid leukemia (AML).

For CMML, the most common monochemotherapy is hydroxyurea, the dose of which is adjusted depending on the number of leukocytes. Comparable results were obtained with therapy with 6-mercaptopurine. However, complete remissions are not achieved with this therapy.

Atypical chronic myeloid leukemia

"Atypical chronic myeloid leukemia" (aCML) differs from classical CML in the absence of the Ph chromosome and the chimeric BCR/ABL gene. In addition, aCML is associated with significant granulocytic, often multilineage dysplasia, which is not observed in CP CML.

The disease has an aggressive course. The average life expectancy is 11-18 months. Differential diagnosis should be carried out primarily with CML. Therapy is almost identical to that for CML.

Juvenile myelomonocytic leukemia

Juvenile myelomonocytic leukemia (jMML) is a hematopoietic clonal disease, characterized by the predominant proliferation of neutrophil and monocyte lines, the absence of the Ph chromosome and the BCR/ABL fusion gene.

In this case, the presence of mutations in the RAS gene family, responsible for the response to growth factors, was noted; mutations of the PTPN11 gene, and the NF1 gene, responsible for the reverse regulation of the activity of the RAS gene. These mutations allow the growth of myeloid progenitors in the bone marrow without the addition of growth factors.

Examination in most cases reveals hepatosplenomegaly and lymphadenopathy. Diagnosed more often in children of early and adolescence, although faces can also hurt young. Differential diagnosis must be carried out with childhood CML and CMML. The jMML category includes individuals with Xp7 monosomy. Therapy is carried out according to generally accepted protocols for the treatment of CMML with the addition of retinoids. Cure is possible only with allogeneic transplantation bone marrow(allo-BMT).

Chronic neutrophilic leukemia

Fewer than 150 cases have been described in the literature of this disease, but in most cases the diseases were associated with the presence of another pathology, in particular myeloma.

Later observations, in the absence of cytogenetic changes, suggested that neutrophilia is due to abnormal cytokine production in the presence of a tumor or an abnormal inflammatory response. Clinical and laboratory data may correspond to those of CML in CP.

However, in some cases, cytogenetic and molecular studies have proven the clonality of the neutrophil line. Therefore, chronic neutrophilic leukemia is included in the cMPN group according to the WHO classification with a recommendation to confirm the clonal nature of myeloid metaplasia by cytogenetic study data in the presence of other tumor diseases. Therapy, if clonality is proven, is carried out in the same way as therapy for CML in the corresponding phase.

Chronic eosinophilic leukemia, unspecified (CEL NS)

Cases of myeloproliferative neoplasms in the absence of PDGFRA, PDGFRB or FGFR1 rearrangement are classified as CEL NS in the presence of blood eosinophilia >1.5x10 9 /L or more, the number of blasts in the peripheral blood is less than 20%, the absence of the BCR-ABL1 fusion gene, the presence of inv (16) (p13.1; q22) or t (16; 16) (p13.1; q22), proliferation of eosinophils in bone marrow (BM) and tissues various organs(heart, lungs, skin, central nervous system, gastrointestinal tract).

Confirmation of the clonality of leukemic cells in some cases are karyotype abnormalities: +8, monosomy 7, aberration of chromosomes 4, 6, 10, 15, as well as JAK2 mutation; there is no clonality of T-cell receptors.

Differential diagnosis carried out with various reactive eosinophilias, hypereosinophilic syndrome (HES) and tumor diseases with an increase in the number of eosinophils (Hodgkin lymphoma, acute lymphoblastic leukemia and CML). Previously, HEL NS and HPP were combined into one nosological group.

In the present classification, an increase in the number of blasts in the blood >2%, in the BM >5% and confirmation of the clonality of proliferating cells makes it possible to separate these two pathological conditions. Therapy is carried out according to the rules for the treatment of CML with the obligatory prescription of disaggregant therapy due to the presence of a tendency to hypercoagulation and possible thrombosis of mesenteric vessels.

WHO classification of mast cell diseases

WHO (2008) classification of mast cell diseases:

Cutaneous mastocytosis;
- indolent systemic mastocytosis;
- systemic mastocytosis associated with a clonal hematological disease of a non-mast cell lineage;
- aggressive systemic mastocytosis;
- mast cell leukemia;
- mast cell sarcoma;
- extracutaneous mastocytoma.

The term mastocytoses encompasses a group of proliferating mast cell diseases characterized by abnormal proliferation and accumulation of mast cells in one or more organ systems.

Hematological changes in mastocytosis include anemia, leukocytosis with eosinophilia, granulocytopenia and thrombocytopenia. CM is affected in patients with the aggressive or leukemic variant. In the trephine, when the bone marrow is damaged, multifocal clusters or infiltration by mast cell aggregates are detected; histological examination reveals diffuse interstitial infiltration.

Cutaneous mastocytosis, or urticaria pigmentosa, occurs predominantly in children and manifests itself as small papular, urticarial, bullous and diffuse pinkish rashes on the skin.

Systemic mastocytosis is observed more often in adults and is characterized by abnormal infiltration mast cells not only the skin, but also the bone marrow, spleen, gastrointestinal tract and others internal organs. In some patients, systemic mastocytosis is associated with the development of chronic MPN, less often - MDS or mature cell lymphoid proliferation.

In the clinical picture of systemic mastocytosis, two groups of symptoms are distinguished. Symptoms of the first group are caused by the infiltration of organs and tissues by mast cells. Symptoms of the second group include: intoxication, itching, osteoporosis or osteofibrosis, diarrhea and ulcerative lesions of the gastrointestinal tract, hemorrhagic syndrome.

The clinical variant of systemic mastocytosis, which occurs with massive damage to the bone marrow (more than 20% of mast cells) and the appearance of abnormal mast cells in the blood, is designated as mast cell leukemia. This option is characterized by the absence skin lesions and unfavorable course.

Diagnosis of mastocytosis is based on identifying mast cell infiltration of affected organs and tissues. To clarify the diagnosis, immunophenotypic determination of CD2, CD 25, tryptase (G3) or determination of the c-kit mutation (CD 117) is performed.

Differential diagnosis of reactive mast cell hyperplasia against the background of allergic and tumor diseases is based on morphological data.

Treatment uses production inhibitors and antagonists of mediators released from mast cells. There are reports of positive results from the use of interferon and

More than 90% of patients are men, usually older. WHO classifies hypereosinophilic syndrome as a myeloproliferative disorder, recognizing that not all cases arise at the stem cell level. It can be almost impossible to distinguish clonal proliferation of eosinophils from reactive ones caused by causeless excessive production of cytokines. If there are no signs of clonality (eg, chromosomal abnormalities), a diagnosis of hypereosinophilic syndrome is made; otherwise, eosinophilic leukemia is diagnosed.

The etiology of hypereosinophilic syndrome is unknown. It is assumed that GM-CSF, IL-5 and IL-7 are responsible for the excessive formation of eosinophils. Despite the pronounced tendency to thrombosis, no specific disorders were found in the coagulation and fibrinolytic systems.

Damage to internal organs:

Hematopoietic disorders. The absolute number of eosinophils usually ranges from 3000 to / μl; The diagnosis is made if the eosinophil count exceeds 1/µL for 6 months or longer and there are no other causes of eosinophilia. Eosinophils are usually small mature cells with a reduced number of granules. In half of the patients, normocytic normochromic anemia is detected. In the bone marrow, the number of myeloid cells is increased, 25-75% of them are eosinophils with an increase in the number of immature elements. The content of myeloblasts is not increased, there are no chromosomal abnormalities.

Damage to the nervous system (40-70% of cases) is manifested by cerebral embolism, encephalopathy and sensory neuropathy. Only nonspecific changes are detected in biopsy specimens.

Lung involvement (40-50% of cases) usually manifests itself over a long period of time unproductive cough. In the absence of heart failure and pulmonary embolism, pulmonary function tests are unchanged. On radiographs, focal or diffuse lung damage is detected in only 20% of patients. Bronchial asthma with hypereosinophilic syndrome is rare.

Other myeloproliferative diseases. Hypereosinophilic syndrome is rarely accompanied by severe myelofibrosis and hyperplasia of other cell lineages.

Eosinophilia with damage to individual organs is not accompanied by multiple organ damage, often observed in hypereosinophilic syndrome.

III. Diagnostics. Criteria for hypereosinophilic syndrome:

1. Persistent eosinophilia more than 1500 μl"6 for 6 months.

2. Absence of helminthiases, allergic reactions and other causes of eosinophilia.

3. Signs of damage to internal organs.

4. Absence of chromosomal abnormalities (otherwise the diagnosis of eosinophilic leukemia is made).

Detailed history and physical examination, complete blood count, liver and kidney function tests, urinalysis.

IgE level and serological tests for collagenoses.

Chest X-ray.

Cytological, histological and cytogenetic examination of bone marrow.

Biopsy of skin lesions.

Repeated stool examinations for helminths and their eggs.

Examination of duodenal contents and serological testing for strongyloidiasis.

Inoculation on media for bacteria, mycobacteria and fungi.

IV. Forecast. More than 75% of patients survive 5 years, and 40% survive 10 years or longer, depending on the success of treatment of lesions of internal organs. The prognosis is unfavorable for refractory heart failure and leukocytosis above / µl.

V. Treatment. As long as there are no signs of damage to internal organs, therapy should be abstained. Glucocorticoids are the most effective. When organ function is restored and the number of eosinophils decreases to upper limit normal treatment is stopped. If prednisone is ineffective, monochemotherapy with hydroxyurea, vincristine or chlorambucil is prescribed. Polychemotherapy should be avoided. Leukapheresis is useless, since the level of eosinophils returns to baseline within 24 hours. Antiplatelet drugs (aspirin) or anticoagulants (warfarin) are often prescribed, but their effectiveness has not been proven.

Hypereosinophilic syndrome, literature:

Bain B.J. Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome. Br J Haematol 1996; 95:2.

Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood 2002; 100:2292.

Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994; 83:2759.

Autoimmune symptoms

It is currently debated whether there are significant differences between ES and acute eosinophilic leukemia (AEL). No agreement has been reached, but there appear to be some overlapping symptoms. Two types of clinical outcome and degrees of visceral involvement are observed. In patients with symptoms of primary damage to the heart and lungs, as well as vasculitis, a subsequent clinical course most likely corresponds to disseminated vasculitis. On the other hand, patients with primary hepatoeplenomegaly, unusually high blood eosinophil counts, and rapid clinical deterioration appear to have malignant ALE.

ES is very rare in children. Variants of the disease may be observed, which are characterized by isolated damage to certain organs. The primary cardiac lesion that is diagnosed as endocarditis with endomyocardial fibrosis may actually be a variant of ES.

Other autoimmune conditions that occur with eosinophilia are also known: eosinophilic fasciitis, rheumatoid arthritis, periarteritis nodosa, chronic hepatitis, regional enteritis, eosinophilic cystitis, eosinophilic gastroenteritis and infected gastroperitoneal shunt (see table). There is agreement on the issue that eosinophilia in chronic peritoneal dialysis is of an autoimmune nature. A full discussion of each of the clinical options is beyond the scope of this chapter; some of them are mentioned below in context. differential diagnosis for patients who have a symptom of eosinophilia.

In addition to systemic autoimmune diseases, eosinophilia can be observed during local inflammatory processes, which can be difficult to distinguish from the early stages of ES. Some of these immune diseases are described below.

Eosinophilic fasciitis. Eosinophilic fasciitis is a disease that affects the face and skin and is difficult to distinguish from scleroderma. It differs from scleroderma in its relatively acute onset, appearance after unusual physical activity, and sensitivity to corticosteroid hormones. Eosinophilia is usually found in the blood and skin tissue. In contrast to scleroderma, in the pathogenesis of the inflammatory process in eosinophilic fasciitis, degranulation of mast cells is more important than deposits of immune complexes.

Eosinophilic gastroenteritis. Apparently, in eosinophilic gastroenteritis, an autoimmune mechanism of activation of the complement cascade by mast cells operates. Patients present with symptoms such as afternoon nausea, vomiting, cramps, periumbilical pain, and loose, watery stools. Charcot-Leyden crystals, which are products of eosinophil degradation, may be present in the stool. Rectoscopy or rectosigmoid biopsy often reveals thickening of the intestinal wall. The pathogenesis of this disease is not fully understood, but there is strong evidence in favor of an autoimmune mechanism for its development.

Eosinophilic cystitis. Inflammation of the bladder is known to resemble other forms of intractable cystitis, such as interstitial cystitis due to tuberculosis and bladder neoplasms, which occurs secondary to allergic or immune disorders. A constant feature is eosinophilia in the blood and bladder wall. The disease usually has a chronic course and in some patients is caused by food allergens.

Hepatitis. Hepatitis can also occur with eosinophilia. In most cases, the possibility of hepatitis is indicated general symptoms and signs, with isolated eosinophilia without such symptoms it is difficult to make a correct diagnosis.

Eosinophilia in malignant neoplasms

It is known that eosinophilia can be a symptom accompanying various tumor lesions. Most often, eosinophilia is observed in combination with cancer of the nasopharynx and bronchial tubes, as well as with adenocarcinoma of the stomach, colon, uterus and thyroid gland. In addition, it is observed in Hodgkin's disease and histiocytoma.

In all likelihood, eosinophilia in malignant neoplasms has clinical significance. It has been noted that eosinophilia can be observed in tumor tissue and in the blood. Tumors in which isolated eosinophilia is observed in the neoplasm tissue have a more favorable prognosis than those without eosinophilia. However, often tumors that occur with eosinophilia in the blood spread quickly and have an unfavorable prognosis.

Malignant transformation of eosinophils is observed in AEL (Fig.). However, eosinophilia is not uncommon in other leukemias, such as acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). It can sometimes be very difficult to differentiate AEL from reactive eosinophilia associated with ALL or AML. The most definitive answer to distinguishing between these types is provided by studies of special cell markers. The difficulties in differentiating ES from eosinophilia associated with leukemia have been discussed previously. In this regard, it is important to note that chloromas and blastomas (i.e., accumulations of eosinophils and blast cells) are very rare in ES, but are often observed in leukemia. In addition, when comparing patients with ES, in whom chromosomal studies were performed, with carpotypes of patients with a confirmed diagnosis of leukemia, it was found that ES is usually not accompanied by any disorders, unlike ALL, AML AEL, in which aneuploid and polyploid changes are observed. Due to this, karyotype studies can be critical in differentiating these conditions.

Bone marrow preparation from a patient with acute eosinophilic leukemia.

Blast forms of eosinophils infiltrating the bone marrow dominate over other cell types.

Eosinophilia in pediatric practice

Eosinophilic gastroenteritis is predominantly a disease of childhood, and most patients are under 20 years of age. An allergic history is not always available.

Eosinophilia usually occurs in premature infants and persists until they reach normal body weight. Currently, a high eosinophil count is considered a sign of an anabolic state.

Acute myeloid leukemia - symptoms of promyelocytic, monoblastic, myelomonocytic myeloid leukemia

The concept of acute myeloblastic (or myeloid) leukemia (abbreviated as AML) combines several types of cancer hematopoietic system a person in which the bone marrow becomes the focus of cancer

Until today, oncohematologists do not have a single confidence in the exact causes of disruption of the hematopoietic system, so it is quite difficult to identify special risk groups, and even more so to predict the likelihood of developing myeloid leukemia, or blood cancer. Science makes every effort to create effective methods diagnosis and treatment of AML, as a result of which acute myeloid leukemia, diagnosed in the early stages, now has a favorable prognosis for survival.

How does myeloid leukemia develop?

If you imagine the role of the bone marrow as a producer of the entire variety of blood cells, then myeloid leukemia will look like a kind of sabotage in this well-functioning production.

The fact is that disruption of the bone marrow in myeloid leukemia is accompanied by the release into the blood production system of a huge number of “immature” or underdeveloped white blood cells - myeloblasts - leukocytes that have not yet acquired their immune function, but at the same time began to multiply uncontrollably. As a result of such a mutation, the coordinated process of regular renewal of leukocytes in the blood is disrupted and the rapid displacement of full-fledged blood cells by abnormal precursor cells begins. In this case, not only leukocytes are displaced, but also red blood cells (erythrocytes) and platelets.

Types of myeloid leukemia

Due to the fact that the mutation of blood cells itself rarely develops in the body in a “pure” form, but is most often accompanied by other mutations of stem cells and other pathologies, there are many various forms and types of myeloid leukemia.

If until recently there were 8 main types, divided according to the origin of leukemic formations, today mutations that have occurred in cells at the genetic level are also taken into account. All these nuances affect the pathogenesis and prognosis of life expectancy in one form or another of the disease. In addition, determining the type of acute myeloid leukemia disease allows you to select an appropriate treatment regimen.

According to the FAB, variants of myelocytic lecosis are divided into the following subgroups:

Features of acute promyelocytic leukemia

APL, or APML, which stands for acute promyelocytic leukemia, is a subtype of myeloid leukemia M3 according to the FAB (French-American-British classification). With this malignant disease, an abnormal number of promyelocytes, which are immature granulocytes, accumulate in the blood and bone marrow of patients.

Acute promyelocytic leukemia is defined by a typical chromosome translocation, leading to the formation of abnormal oncoproteins and uncontrolled division of mutated promyelocytes. It was discovered in the middle of the 20th century and for a long time was considered one of the fatal and ultra-acute forms of myeloid leukemia.

Currently, acute promyelocytic leukemia shows a unique response to treatments such as arsenic trioxide and trans-retinoic acid. Thanks to this, APL has become one of the most favorably predicted and curable subtypes of the disease acute myeloid leukemia.

The life expectancy forecast for this variant of AML in 70% of cases is 12 years without exacerbations.

Promyelocytic leukemia is diagnosed by bone marrow tests, blood tests and additional cytogenetic studies. The most accurate diagnostic picture can be obtained through PCR (polymerase chain reaction) research.

Characteristics of acute monoblastic leukemia

Acute monoblastic leukemia belongs to the interregional form of AML in accordance with the FAB classification - variant M5, which occurs in 2.6% of cases in children and in 6-8% of cases in adults (most often the elderly).

Indicators clinical picture practically do not differ from acute myeloid leukemia, although the general symptoms are supplemented by more severe intoxication and high temperature bodies.

The disease is also characterized by signs of neutropenia with a predominance of necrotic changes in the nasopharyngeal mucosa and oral cavity, as well as inflammation of the tongue.

The main site of localization of the disease is the bone marrow, but an enlargement of the spleen and certain groups of lymph nodes is also observed. In the future, infiltration of the gums and tonsils, as well as metastasis of the tumor to the internal organs, may occur.

However, with timely testing, detection of malignant pathology and the use of modern treatment regimens, in 60% of cases a significant improvement in the patient’s condition is predicted.

Characteristics of eosinophilic leukemia

Acute eosinophilic leukemia develops as a result of malignant transformation of eosinophils and can occur against the background of adenocarcinoma of the thyroid gland, uterus, intestines, stomach, bronchial and nasopharyngeal cancer. This type of myeloid leukemia is similar to the reactive eosinophilia inherent in acute lymphoblastic leukemia (ALL) or myeloblastic leukemia. Therefore, to differentiate diagnostics, they resort to studies of specific cellular blood markers.

The most characteristic features of this subtype of myeloid leukemia are an increase in the number of eosinophils and basophils in a blood test, and an increase in the size of the liver and spleen.

Features of myelomonocytic leukemia

Of particular concern to modern oncohematologists is such a subgroup of AML as myelomonocytic leukemia, varieties of which most often affect children. Although among the elderly population the risk of contracting this type of myeloid leukemia is also high.

Myelocytic leukemia is characterized by an acute and chronic course, and one of the chronic forms is juvenile myelomonocytic leukemia, characteristic of children from the first year of life to 4 years. The peculiarity of this subtype is the frequency of its development in young patients and the greater predilection for the disease in boys.

Why does myeloid leukemia develop?

Despite the fact that the exact causes of leukemia have still not been established, in hematology there is a certain list of provoking factors that can have a destructive effect on the activity of the bone marrow:

radiation exposure;
unfavorable environmental living conditions;
work in hazardous production;
influence of carcinogens;
side effects from chemotherapy for other forms of cancer;
chromosomal pathologies – Fanconi anemia, Bloom and Down syndromes;
the presence of pathologies such as Epstein-Barr virus, lymphotropic virus or HIV;
other immunodeficiency conditions;
bad habits, especially smoking, of parents of a sick child;
hereditary factor.

How does myeloid leukemia manifest?

Due to the fact that the symptoms of myeloid leukemia vary depending on the forms and types of AML, the allocation of general clinical indicators to the category of symptoms is very arbitrary. As a rule, the first warning signs are detected in the results of a blood test, which forces the doctor to prescribe additional methods diagnostics

AML in children

In the case of young children, who are most susceptible to a type of juvenile myelomonocytic leukemia, the presence the following symptoms should alert parents and force them to see a doctor:

If the child is not gaining weight well;
If there are delays or deviations in physical development;
Increased fatigue, weakness, pale skin due to iron deficiency anemia;
Presence of hyperthermia;
Frequent infections;
Enlarged liver and spleen;
Swelling of peripheral lymph nodes.

Of course, the presence of one or more of the above symptoms does not mean that the child is definitely developing juvenile myelocytic leukemia, because such indicators are characteristic of many other diseases. But, as you know, treatment of complex diseases is most effective in the early stages, so take blood tests and undergo other diagnostic procedures will not be superfluous.

AML in adults

chronic fatigue, general weakness;
loss of weight and appetite;
tendency to internal hemorrhages, bruising, increased bleeding;
increased bone fragility;
frequent dizziness and chills;
instability to infectious pathologies;
nausea;
constant pallor.

It is clear that these symptoms cannot serve as the only factor in determining AML, so you should not self-diagnose cancer.

Diagnostic procedures for AML

The first and fundamental diagnostic measure To verify myeloid leukemia, a complete blood test is used. If a pathological proliferation of certain groups of blood cells is detected, a bone marrow biopsy is prescribed. To determine the distribution cancer cells used in the body:

X-ray and ultrasound examinations;
skeletal scintigraphy;
computed and magnetic resonance imaging.

As a rule, all diagnostic procedures are carried out in hematology and oncology clinics, and when the diagnosis of AML is confirmed, a treatment plan is immediately drawn up. Since pathogenesis (course) different forms The disease differs at the cellular and molecular level, the prognosis of the patient’s life expectancy depends entirely on the accuracy of the diagnosis and the adequacy of the chosen treatment method.

Therapeutic measures

Today, treatment of myeloid leukemia consists of 4 stages of therapeutic measures:

Induction with intensive use of chemotherapy designed to destroy as many myeloid cells as possible in the shortest possible time to achieve a remission period.
Consolidation with intensive therapy of combined and additional chemotherapy doses to destroy remaining tumor cells and reduce the risk of disease return.
Treatment of the central nervous system, carried out to prevent leukemia cells from reaching the spinal cord and brain, to prevent metastasis. If leukemia cells fall into the central nervous system, a course of radiation therapy may be prescribed.
Long-term maintenance therapy prescribed for a long period(a year or more) and carried out on an outpatient basis with the aim of destroying surviving cancer cells.

Side effects of chemotherapy

Despite the effectiveness of chemotherapy treatment, not every patient agrees to use high doses of chemotherapy, since this technique has a significant drawback - side complications.

Most common side effect cytopenia is considered to develop as a result of a violation of the hematopoietic process (myelotoxicity). Great danger represents the occurrence of leukopenia, since due to a lack of white blood cells the body loses immune protection from infectious diseases associated with danger to life.
No less of a problem (sometimes even fatal) are thrombocytopenia and iron deficiency anemia confirmed by tests, in the fight against which the body is sometimes oversaturated with iron elements, which leads to secondary changes in internal organs.
Taking cytostatics leads to nausea and vomiting, which is extremely poorly tolerated by patients. Prolonged vomiting leads to dehydration and electrolyte imbalance, anorexia (complete loss of appetite) and even stomach bleeding.
A common side effect is alopecia (baldness), damage to the kidneys and heart muscle, jaundice, ulceration of the mucous membranes and other symptoms. side complications, which occur depending on the patient’s age, stage of the disease, combination of drugs and other factors.

Is it possible to defeat leukemia?

Today it is too early to talk about a complete victory over leukemia. But an increase in life expectancy after intensive therapy by at least 5-7 years is noted on average in 60% of patients. True, the forecasts for patients over 60 years of age do not rise above a 10% indicator. Therefore, you should not wait until you reach old age in order to take serious care of your own health. Pass preventive examinations, monitor your diet and lifestyle, and donate blood and urine tests regularly.

eosinophilic leukemia

Russian-Italian medical dictionary with indexes of Russian and Latin terms. - M.: “Russo”. C.C. Prokopovich. 2003.

See what “eosinophilic leukemia” is in other dictionaries:

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Definition and clinical picture

Hypereosinophilic syndrome is manifested by high eosinophilia in the blood and bone marrow, as well as infiltration of internal organs by relatively mature eosinophils. More than 90% of patients are men, usually aged 20-50 years. WHO classifies hypereosinophilic syndrome as a myeloproliferative

tive diseases, recognizing that not all cases arise at the stem cell level. It can be almost impossible to distinguish clonal proliferation of eosinophils from reactive ones caused by causeless excessive production of cytokines. If there are no signs of clonality (for example, chromosomal abnormalities), put Diagnosis, -a; m. A brief medical report about the disease and condition of the patient, made on the basis of anamnesis and a comprehensive examination. From Greek - recognition, diagnosis, -and; and. 1. A set of techniques and methods, including instrumental and laboratory ones, that allow one to recognize the disease and establish a diagnosis. From Greek - capable of recognizing. 2. Diagnosis, dialysis, -a; m. peritoneal dialysis. A method for correcting water-alectrolyte and acid-base balance and removing toxic substances from the body when a dialysate solution is introduced into the abdominal cavity.

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Heart damage (55-75% of cases). Biopsy specimens reveal foci of myocardial necrosis and an increased number of eosinophils in the endocardium. Parietal thrombi in the cavities of the heart can be a source of thromboembolism. Approximately 2 years after the onset of eosinophilia, endomyocardial fibrosis develops with mitral and tricuspid insufficiency and restrictive cardiomyopathy.

Damage to the nervous system (40-70% of cases) is manifested by cerebral embolism, encephalopathy and sensory neuropathy. Only nonspecific changes are detected in biopsy specimens.

Lung involvement (40-50% of cases) usually manifests as a prolonged, non-productive cough. In the absence of heart failure and pulmonary embolism, pulmonary function tests are unchanged. On radiographs, focal or diffuse lung damage is detected in only 20% of patients. Bronchial asthma with hypereosinophilic syndrome is rare.

Damage to the skin and mucous membranes - Urticaria; and. An allergic disease accompanied by the appearance and disappearance of itchy blisters on the skin, similar in appearance to nettle burns.

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Damage to other organs. In 40% of patients the spleen is enlarged. Arthralgia and effusion occur. Fluid that leaks from small blood vessels into tissues or body cavities due to inflammation or swelling

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rockolitis, chronic active hepatitis. Inflammatory liver disease caused by inf. agents, certain medications, industrial and other poisons; accompanied by jaundice, stool discoloration, hemorrhagic rash, and sometimes nosebleeds. > From Greek. hepar (hcpatos) - liver.

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A. Eosinophilic leukemia differs from hypereosinophilic syndrome by an increased content of blasts in the bone marrow and chromosomal abnormalities.

B. Other myeloproliferative diseases. Hypereosinophilic syndrome is rarely accompanied by severe myelofibrosis and hyperplasia of other cell lineages.

B. Other hemoblastoses, especially acute myelomonoblastic leukemia with eosinophilia, T-cell lymphomas, Lymphogranulomatosis, -a; m. A form of lymphoma (tumors of the lymphatic system), in which special malignant cells (Reed-Berezovsky-Sternberg cells) are produced in the lymph nodes; As a rule, it develops after 10 years of age, the peak incidence is 20-29 years and after 55 years, more often in men. Syn: Hodgkin's disease.

D. Eosinophilia with damage to individual organs is not accompanied by multiple organ damage, often observed in hypereosinophilic syndrome.

D. Churg-Stroe syndrome is a systemic vasculitis. Inflammation of small blood vessels, usually with inf. and inf.-allergic. diseases (for example, rheumatism, sepsis, typhus, etc.), manifested by small hemorrhagic rashes (with damage to the vessels of the skin), abdominal pain (with damage to the vessels of the abdominal organs), etc. o From Lat. vasculum - vessel.

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EOSINOPHILIC LEUKEMIA

(l. eosinophilica) chronic myeloid leukemia, the morphological substrate of which is represented predominantly by acidophilic granulocytes (eosinophils).

Medical terms. 2012

Symptoms

On early stage leukemia, the following may occur:

weakness and soreness;
increase in the size of the spleen;
enlarged lymph nodes in groin area, in the armpits, on the neck.

As the disease progresses, symptoms change. Like the process itself, they become more complex and painful.

Later the following symptoms may appear:

rapid and irresistible fatigue;
dizziness;
high body temperature;
sweating, especially night sweats;
bleeding gums;
anemia;
heaviness in the hypochondrium;
rapid weight loss;
loss of appetite;
liver enlargement.

The last stages are characterized by frequency infectious diseases and the appearance of thrombosis.

Diagnostics

For a complete and deep diagnosis, modern medicine represents a whole process using different methods and directions. All of them are based on laboratory research.

To begin with, a blood test is performed, because it is this procedure that shows the complete picture of the patient’s disease.

The diagnosis is based on this information. In chronic leukemia, the number of platelets and red blood cells is low, while the number of white blood cells is higher than normal.

After biochemical analysis, the blood picture will show all the details about the malfunction different organs and their systems. Next, a puncture is performed.

This process is formed in two stages:

bone marrow puncture. Based on the results of this procedure, the diagnosis and possible methods of treatment are confirmed.
puncture spinal cord . This process helps to identify tumor cells, because they quickly spread specifically to cerebrospinal fluid. After receiving the result, a chemotherapy treatment program is formed, because the characteristics of the body and the effect of the disease on it differ in patients.

Depending on the type of disease, the following may be performed: immunocytochemistry, genetic studies, cytochemistry, X-ray studies(in case joints and bones are damaged by leukemia), CT scan(for analysis of lymph nodes abdominal cavity), MRI (examination of the spinal cord and brain), ultrasound

Classification

Myelomonocytic

Myelomonocytic leukemia is one of the types of myelomonoblate leukemia, in which blast cells can form the basis for granulocytes or monocytes.

This type is more common in children and older people.

With this disease, anemia manifests itself more clearly. Constant fatigue, pallor and intolerance of the body to physical activity, bleeding and bruising are the main characteristics. Also, myelomonocytic leukemia can lead to neuroleukemia (a disorder of the central nervous system).

Myeloblastic

The reason for the appearance is a DNA defect in bone marrow cells that have not yet matured. At the same time, modern doctors cannot name the main reason, since the occurrence of this disease can often be a consequence of radiation, benzene poisoning, pollution external environment. This type can occur in people of different ages, but him acute form occurs more often in adults.

Myeloid leukemia leads to the uncontrolled development of immature cells that can no longer function stably. Wherein mature cells blood of all types decreases. This type is divided into its subtypes.

Monocytic

Monocytic leukemia is a tumor process in which the number of monocytic cells increases. main feature This type is anemia, which can be the only symptom for a very long time. Often this type of leukemia occurs in people over 50 or in children in their first year of life.

Disseminated intravascular coagulation sometimes occurs in patients with monocytic leukemia. In this case, there is almost no enlargement of the liver and lymph nodes, but the size of the spleen may increase.

Megakaryocytic

Megakaryocytic leukemia is a type of leukemia where the blast cells are megakaryoblasts. This species is quite rare. It is often called "hemorrhagic thrombocythemia", but bleeding is not always found in patients. More often it is characterized by extremely active blood thrombocytosis.

Common in children with Down syndrome, children under 3 years of age, and adults.

The myelocytic lineage is characterized by clonality. Therefore, the spleen often enlarges, bleeding in the gums, nosebleeds, pallor and severe fatigue, shortness of breath, low resistance to infectious diseases, and bone pain.

Eosinophilic

The name of the type itself says that eosinophilic leukemia is accompanied by a process of increasing the number of eosinophils. Hypereosinophilic syndrome, bronchial asthma, urticaria, dermatosis, eosinophilic bone granuloma can cause this disease. In children, eosinophilic leukemia occurs with high body temperature, an increase in the number of leukocytes and eosinophils in the blood, and an increase in the size of the spleen and liver.

Lymphatic

Lymphatic leukemia – cancer, in which lymphatic tissue is affected. The tumor develops very slowly, and the process of hematopoiesis can only be disrupted by late stages. This type of leukemia occurs most often in people over 50 years of age.

The first symptom is enlarged lymph nodes.

The spleen also becomes quite enlarged. General weakness, frequency of infectious diseases and sudden weight loss are also symptoms of lymphatic leukemia.

Treatment of chronic leukemia

The question arises: chronic leukemia - does the disease go away or not?
Treatment of leukemia is carried out depending on its type, risk group and phase.
Groups are determined based on cellular changes, distribution of the disease process, and symptoms. People at low risk are not given sequential treatment. They are subjected to careful observation. But in case of complications or progression of leukemia, treatment is determined to be extremely necessary.

Patients with intermediate or high group Risks without obvious symptoms are also not subject to treatment. Therapy is prescribed only when symptoms of the disease develop.

Chemotherapy is carried out using Chlorambucil.

If side effects occur in a patient, this antitumor drug can be replaced with Cyclophosphamide. Steroid drugs are used less frequently. Some patients undergo combination chemotherapy.

Antitumor drugs, used in different combinations, destroy tumor cells.

Chemotherapy is divided into two stages:

induction therapy. This stage is very intense during the 4-6 week period. If treatment is not continued, the remission caused by induction therapy may disappear;
consolidation therapy. It is aimed at destroying pathological cells. During this period, the patient takes medications that reduce the body’s resistance to therapy.

Bone marrow transplantation plays an important role in the treatment of chronic leukemia.

The produced cells are destroyed by radiation, and new ones are introduced along with healthy cells from the donor. Today, a new technique is popular - bioimmunotherapy using monoclonal antibodies, in which tumor cells are destroyed without damaging healthy tissue.

Forecast

For myelomonocytic leukemia the outcome of the disease is often favorable, 60% of children recover.

People who have myeloblastic leukemia without proper treatment they die. But modern equipment and techniques can give a chance of survival depending on its variety, age and general condition. Now 50-60% recover. Survival statistics for older people are much worse.

Lymphoid leukemia quite difficult to treat. The main stages depend on the classification of this type. But in general, 60-70% of patients recover.

Monocytic leukemia treatable, but quite difficult. When using polychemotherapy or bone marrow transplantation, the chances of survival increase.

Megakaryocytic leukemia is one of the heaviest types. Almost half of pediatric patients do not survive. In adults, the number of people cured is much lower. In children with Down syndrome, this form of leukemia is almost always treatable.

Patients who have been diagnosed lymphatic leukemia with the correct selection of treatment methods, they live on average 5-6 years, sometimes even 10-20, but as a result they die from pneumonia, anemia, and sepsis. Lymphocytic leukemia is rare in adults, but accounts for half of the leukemias in children. This type is treatable.