Chronic lymphatic leukemia. It is a monoclonal neoplasm of lymphocytes, usually B, immunologically defective. It is considered a form of non-Hodgkin’s lymphoma of low aggressiveness, with medullary and peripheral blood involvement (hence the term leukemia).
Summary
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- 1 Etiology
- 2 Staging of chronic lymphatic leukemia
- 1 Rai system
- 2 International system
- 3 Lymphoid areas
- 3 Clinic
- 4 Treatment
- 5 Tricholeukemia, hairy cell leukemia or leukemic reticuloendotheliosis.
- 6 Treatment
- 7 Source
Etiology
Its etiology is unknown and constitutes 25% of leukemias, being the most frequent form of chronic leukemia in western countries and the most frequent form of leukemia in the elderly. It usually manifests in people between the ages of 50 and 70 and presents as a leukocytosis with absolute lymphocytosis of small size and normal morphology.
In more than 95% of cases, the lymphocytes of chronic lymphatic leukemia are of B origin, although they have a lower concentration of surface immunoglobulins than normal, have little response to mitogens, and almost never secrete immunoglobulin into the blood. For these reasons, despite the fact that the patient has a large number of B lymphocytes, clinically it is characterized by humoral immunodeficiency.
A cytological feature of chronic lymphatic leukemia cells is the presence of so-called Gumprecht spots and the presence of positive CD5, CD20 and CD19 markers.
Staging of chronic lymphatic leukemia
We have said that chronic lymphatic leukemia is a form of non-Hodgkin’s lymphoma. If we used the staging classification of lymphomas for chronic lymphatic leukemia, we would find that due to the chronic involvement of chronic lymphatic leukemia, all chronic lymphatic leukemias would be stage IV. This has led to the use of specific staging forms for this disease, of which there are mainly two.
Rai system
- Stage 0: absolute lymphocytosis in peripheral blood greater than 15,000 per cubic millimeter.
- Stage I: lymphocytosis plus lymphadenopathy.
- Stage II: lymphocytosis plus liver and / or splenomegaly (with or without lymphadenopathy).
- Stage III: lymphocytosis plus anemia less than 11 g / LD of hemoglobin in men and 10 in women.
- Stage IV: lymphocytosis plus thrombopenia less than 100,000 per cubic millimeter.
International system
- Stage A: leukemia without anemia or thrombopenia , and with less than three lymphoid areas affected.
- Stage B: Leukemia without anemia or thrombopenia, but with three or more lymphoid areas affected.
- Stage C: leukemia with anemia and / or thrombopenia.
Lymphoid areas
Lymphoid areas are considered:
- Cervical and / or supraclavicular lymphadenopathy .
- Axillary lymphadenopathy .
- Groin lymphadenopathy .
- Liver .
- Spleen .
In the latter classification, stage A has a half-life prognosis greater than 10 years, stage B about 7 years, and stage C about 2 years.
Clinic
Initially, patients are asymptomatic, and the diagnosis can be made by a chance finding of peripheral blood lymphocytosis. As the disease progresses, symptoms of anemia, hepatosplenomegaly and organ infiltration, mainly lymphadenopathy, occur. In terminal stages, patients develop thrombopenia and repeated infections as a consequence of humoral immunodeficiency.
Occasionally we can find immunohemolytic anemias or autoimmune thrombopenias, since chronic lymphatic leukemia is a disorder in which immunological phenomena frequently appear.
Manifestation of humoral immunodeficiency is the progressive hypogammaglobulinemia that patients present. Monoclonal gammopathy will almost never appear in the blood, since the cell from which chronic lymphatic leukemia is derived is a cell still immature enough to secrete immunoglobulin into plasma.
As cytogenetic alterations we can find the trisomy of chromosome 12, del (11q) and del (17p), both of poor prognosis and del (13q) which is the most frequent cytogenetic abnormality and has a good prognosis. Unlike chronic myeloid leukemia, where the transformation phase is the rule, transformation into chronic lymphatic leukemia is rare. Most of the patients die from the tumor itself and the situation of humoral immunodeficiency.
In some cases, however, there is a transformation of chronic lymphatic leukemia into another entity, such as large cell lymphoma (highly aggressive lymphoma, generally immunoblastic), a situation that is called Richter syndrome .
Other transformations are the so-called prolymphocytic leukemia (the most frequent), acute lymphoblastic leukemia and multiple myeloma , the latter two very rare.
The prolymphocytic leukemia is usually more common in older people, occurs with a large splenomegaly, with large cells in peripheral blood, and has a worse prognosis.
Treatment
In the asymptomatic phase, observation of patients without treatment is preferable. When the patient begins to develop symptoms, the traditional treatment has been the administration of cytostatics , generally chlorambucil and, less frequently, cyclophosphamide , associated with corticosteroids if they develop immunological processes such as anemia or autoimmune thrombopenia. The multidrug has also been an option.
As modern treatments we find the drugs that are called fludarabine and 2-chlorodeoxydenosine (cladribine), which can produce complete remissions, even in cases resistant to chemotherapy.
Other treatments are monoclonal antibodies (rituximab, alemtuzumab) and transplantation of hematopoietic precursors.
Tricholeukemia, hairy cell leukemia, or leukemic reticuloendotheliosis.
It is a special form of leukemia , usually B, that has many special characteristics. From a morphological point of view, the cells present cytoplasmic projections in the form of hairs, which gives its name to the entity (hairy cell or whipworm). From the cytochemical and immunological point of view, the cells have the characteristic staining for tartrate resistant acid phosphatase and CD 25 marker.
Clinically, these are middle-aged people with pancytopenia (unlike most leukemias with increased blood cells), and progressive splenomegaly until it becomes massive with few lymphadenopathy.
The diagnosis is based on the study of peripheral blood and bone marrow , taking into account that the medullary aspirate is dry, as a consequence of the intense medullary fibrosis accompanying the tumor. For this reason, a biopsy is essential. As special clinical characteristics, it must be remembered that up to 30% of the cases of whipworm cancer are associated with vasculitis, generally of the panarteritis nodosa type, and that Legionella pneumonia appears as a frequent infectious complication.
Treatment
The classic treatment has been splenectomy, since most of the tumor mass is found in the spleen. As new treatments, interferon alfa and pentostatin or deoxychoformycin have been used , which induce complete remissions (pentostatin more often than interferon) and better still, cladribine (2-chlorodeoxyadenosine), the drug of choice today.
