Hematology and Oncology Book

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Hairy Cell LeukemiaAka: Leukemic Reticuloendotheliosis

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  1. Epidemiology
    1. Affects patients over age 40 years
    2. More common in Males
  2. Pathophysiology
    1. B Lymphocyte proliferation
    2. Commonly associated effects
      1. Splenomegaly
      2. Impaired host defense and secondary infection
      3. Vasculitis
  3. Symptoms
    1. Bone pain (e.g. Hip Pain) if bone lesions present
  4. Signs
    1. Defining features
      1. Splenomegaly (75%)
    2. Vasculitis associated findings
      1. Erythema Nodosum
      2. Cutaneous Nodules
    3. Rare findings
      1. Lymphadenopathy
      2. Hepatomegaly
  5. Labs
    1. Complete Blood Count
      1. Pancytopenia
      2. Leukocyte count normal or low
    2. Peripheral Smear
      1. Hairy appearing Leukocytes (cytoplasmic projections)
        1. Size: 15 to 20 um in diameter
        2. Eccentric nucleus with foamy cytoplasm
      2. Tartrate-resistant Acid Phosphatase (TRAP) staining
    3. Bone Marrow Aspiration
      1. Difficult due to reticulin fibrosis
      2. Mononuclear cells replace normal architecture
  6. Differential Diagnosis
    1. Chronic Lymphocytic Leukemia
    2. Waldenstrom's Macroglobulinemia
    3. Acute Leukemia
    4. Aplastic Anemia
  7. Complications
    1. Infection (most common cause of death)
      1. Legionella pneumonitis
      2. Toxoplasmosis
      3. Mycobacterium tuberculosis
      4. Atypical Mycobacterium Disease
      5. Nocardiosis
      6. Pyogenic infection
  8. Management: Leukemia progression
    1. Indications
      1. Pancytopenia
      2. Infections
      3. Massive Splenomegaly
      4. Rapid disease progression
    2. Management Options
      1. Splenectomy
      2. alpha interferon
        1. Highly effective in 70% of cases
        2. Remission is rare
      3. Pentostatin
        1. Very active in hairy cell Leukemia
      4. Glucocorticosteroid Indications
        1. Vasculitis
        2. Autoimmune complications
      5. Chemotherapy with Alkylating Agents contraindicated
  9. Prevention
    1. Pneumococcal Vaccine
  10. Prognosis
    1. Eight year survival >50%

Hairy Cell Leukemia (C0023443)

Definition (MSH)A neoplastic disease of the lymphoreticular cells which is considered to be a rare type of chronic leukemia; it is characterized by an insidious onset, splenomegaly, anemia, granulocytopenia, thrombocytopenia, little or no lymphadenopathy, and the presence of "hairy" or "flagellated" cells in the blood and bone marrow.
Definition (CSP)neoplastic disease of the lymphoreticular cells which is considered to be a rare type of chronic leukemia; it is characterized by an insidious onset, splenomegaly, anemia, granulocytopenia, thrombocytopenia, little or no lymphadenopathy, and the presence of hairy or flagellated cells in the blood and bone marrow.
Definition (NCI)A type of chronic leukemia in which the abnormal white blood cells appear to be covered with tiny hairs when viewed under a microscope.
Definition (NCI)A rare neoplasm of small B-lymphocytes with "hairy" projections in bone marrow, spleen and peripheral blood. Most patients are middle-aged to elderly adults and present with splenomegaly and pancytopenia. (WHO, 2001)
ConceptsNeoplastic Process (T191)
ICD9202.4, 202.4
MSHD007943
EnglishHairy cell leukaemia, Hairy Cell Leukemia, Hairy Cell Leukemias, hairy T cell leukemia, Hairy-cell leukemia, HCL, HCL - Hairy cell leukaemia, HCL - Hairy cell leukemia, Leukaemic reticuloendotheliosis, Leukaemic reticuloendotheliosis -RETIRED-, Leukaemic reticuloendotheliosis of unspecified sites, LEUKEMIA HAIRY CELL, Leukemic Reticuloendothelioses, Leukemic reticuloendotheliosis, Leukemic reticuloendotheliosis -RETIRED-, Leukemic reticuloendotheliosis of unspecified sites, LRE - Leukaemic reticuloendotheliosis, LRE - Leukemic reticuloendotheliosis
Spanishleucemia de celulas pilosas, leucemia de celulas vellosas, leucemia de tricoleucocitos, reticuloendoteliosis leucemica, reticuloendoteliosis leucemica de localizaciones no especificadas, tricoleucemia
Parent Conceptsacquired immunodeficiency (C0596032), viral leukemia (C0597646), Other malignant neoplasm of lymphoid and histiocytic tissue (C0153793), leukemia (C0023418), Lymphoproliferative Disorders (C0024314), Lymphoblastic Leukemia (C0023448), Chronic leukemia (category) (C1279296), Mature B-Cell Neoplasm (C1334633), Hairy Cell Leukemia (C0023443), Leukemia, Lymphocytic, Chronic (C0023458), Diffuse low grade B-cell lymphoma (C0456862), Reason not stated concept (C1276325), Chronic lymphoid leukemia - category (C1531666), Mature (peripheral) B-cell neoplasm (C1532008), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, DXP, ICD9CM, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, NDFRT, PDQ, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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