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Hairy Cell Leukemia (Leukemic Reticuloendothellosis) And Other Variants Of Chronic Myeloid Leukemia

Updated on January 21, 2014

Treatment Of Hairy Cell Leukemia

In the early stages, corticosteroids are useful. The disease responds best to splenectomy.
In the early stages, corticosteroids are useful. The disease responds best to splenectomy. | Source

Hairy Cell Leukemia

This is a variant of chronic lymphatic leukemia and is rare. It is seen in the age group 40-80 years and males are affected four times more common than females. Total leukocyte count may be normal or high. Spleen is grossly enlarged. Blood shows the characteristic hairy cells which have features of B lymphocytes and monocytes. The cytoplasm shows a number of villi which given the hairy appearance. The diagnostic histochemical test for hairy cells is acid phosphatase staining reaction resistant to the action of tartarate. Bone marrow is infiltrated heavily and it is difficult to aspirate and often a dry tap is obtained.

Treatment: In the early stages, corticosteroids are useful. The disease responds best to splenectomy.

Gross Splenomegaly

Prolymphocytic Leukemia is a rare variant of CLL, seen in males above the age of 60 years and showing gross splenomegaly and a very high white cell count, often above 400 X 109/liter.
Prolymphocytic Leukemia is a rare variant of CLL, seen in males above the age of 60 years and showing gross splenomegaly and a very high white cell count, often above 400 X 109/liter. | Source

Leucoerythroblastic patient

Source

Other Variants Of Chronic Myeloid Leukemia

Prolymphocytic Leukemia

This is a rare variant of CLL, seen in males above the age of 60 years and showing gross splenomegaly and a very high white cell count, often above 400 X 109/liter. Lymphadenopathy is rare. The characteristic cell is the large lymphocyte showing a prominent nucleolus. The course is rapidly downhill and prognosis is poor.

Leucoerythroblastic blood picture

In this condition, both erythroid and myeloid precursors are present in peripheral blood. The platelet count may be normal or reduced.

Causes

  1. Metastatic carcinoma involving bone marrow, especially from breast, lung, prostate and thyroid
  2. Myelofibrosis and myelosclerosis
  3. Multiple myeloma
  4. Other miscellaneous conditions are:
  • Hodgkin’s disease
  • Miliary tuberculosis
  • Disseminated intravascular coagulation
  • Hemorrhage
  • Hemolysis
  • Megaloblastic anemia under treatment

The following are the variants of Chronic Myeloid Leukemias

Eosinophilic Leukemias

In this disorder, the patients present with marked eosinophilia often above 100,000/cmm and the clinical picture of chronic myeloid leukemia. The blood shows eosinophils, eosinophil myelocytes and occasional neutrophil myelocytes. In a few cases, Philadelphia chromosomes may be found.

Chronic Basophilic Leukemias

This clinically resembles CML, but there is gross increase in basophils. A few neutrophils may be present. Philadelphia chromosome may or may not be present.

Chronic Monocytic Leukemia

This term is used to denote the condition in which the monocytes are also considerably increased in addition to the blood picture of CML.

Chronic Myelomonocytic Leukemias

This entity which is considered as one of the myelodysplastic syndromes is seen in patients over the age of 50 years and the clinical picture resembles that of RAEB. Blood shows absolute elevation of monocytes (2 X 109/liters or more), many of them being atypical. The spleen enlarges progressively. The main difference from AML (M2, M4, and M5) is that the count of promyelocyte + blasts in the marrow is less than 30%. Over one-third of the cases develop AML on follow up.

These are not common forms of Chronic Myeloid Leukemia, but they still do occur from time to time and for this reason should not be neglected. Even if the incidence is one in a million (of which it could be anyone), the affected person will still need care and attention.

© 2014 Funom Theophilus Makama

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