Hematology and Oncology Book

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Hairy Cell Leukemia

Aka: Hairy Cell Leukemia, Leukemic Reticuloendotheliosis
  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 (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)
Definition (NCI) A rare type of leukemia in which abnormal B-lymphocytes (a type of white blood cell) are present in the bone marrow, spleen, and peripheral blood. When viewed under a microscope, these cells appear to be covered with tiny hair-like projections.
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.
Concepts Neoplastic Process (T191)
MSH D007943
ICD9 202.4
ICD10 M9940/3, M9941/3, C91.4, C91.40
SnomedCT 118613001, 85228003, 190066005, 188653005, 188644003, 54087003
English Hairy Cell Leukemias, Leukemia, Hairy Cell, Leukemias, Hairy Cell, Leukemic Reticuloendothelioses, Leukemic Reticuloendotheliosis, Reticuloendothelioses, Leukemic, Reticuloendotheliosis, Leukemic, hairy T cell leukemia, HAIRY CELL LEUKEMIA, Leukemia, Hairy cell, RETICULOENDOTHELIOSIS, LEUKEMIC, Leukaem reticuloendotheliosis, Leukaemic reticuloendoth. NOS, Leukaemic reticuloendotheliosis NOS, Leukemic reticuloendoth. NOS, Leukemic reticuloendotheliosis NOS, Hairy-cell leukaemia, Hairy-cell leukemia, LEUKEMIA HAIRY CELL, Leukaem.reticuloend.-unsp.site, Leukaemic reticuloendotheliosis of unspecified sites, Leukemic reticuloendotheliosis of unspecified sites, HAIRY CELL LEUKAEMIA, LRE-Leuk reticuloendotheliosis, [M]Leukaem reticuloendothelsis, Leukaem reticuloendothelsis, [M]Leukaemic reticuloendotheliosis, [M]Leukemic reticuloendotheliosis, leukemia hairy-cell, hairy cell leukemia (diagnosis), hairy cell leukemia, Leukemic reticuloendotheliosis (morphologic abnormality), [M]Leukaemic reticuloendotheliosis (disorder), Leukaemic reticuloendotheliosis -RETIRED-, hairy-cell leukemia, Leukemic reticuloendotheliosis of unspecified sites (disorder), Leukemic reticuloendotheliosis NOS (disorder), Leukemia, Hairy Cell [Disease/Finding], cell hairys leukemia, hairy cell leukaemia, hairy-cell leukaemia, leukaemia hairy cell, leukemia hairy cell, cell hairy leukemia, cells hairy leukemia, Hairy cell leukemia NOS, Hairy cell leukemia, Leukemic reticuloendotheliosis, HCL - Hairy cell leukaemia, HCL - Hairy cell leukemia, Hairy cell leukaemia, Leukaemic reticuloendotheliosis, [M]Hairy cell leukaemia, [M]Hairy cell leukemia, LRE - Leukaemic reticuloendotheliosis, LRE - Leukemic reticuloendotheliosis, Hairy cell leukaemia (clinical), Hairy cell leukemia (clinical), Hairy cell leukemia (disorder), Hairy cell leukemia (morphologic abnormality), Leukaemic reticuloendotheliosis (clinical) [obs], Leukemic reticuloendotheliosis (clinical) [obs], Leukemia, hairy cell, leukemia, hairy cell, leukemia; hairy cell, leukemic; reticuloendotheliosis, reticuloendotheliosis; leukemic, Leukemic reticuloendotheliosis -RETIRED-, Hairy Cell Leukemia, HCL
French LEUCEMIE A TRICHOLEUCOCYTES, Leucémie à tricholeucocyte, Leucémie à tricholeucocytes, Réticulo-endothéliose leucémique, Réticuloendothéliose leucémique
Dutch leukemische reticulo-endotheliosis, leukemie; haarcellen, leukemisch; reticulo-endotheliose, reticulo-endotheliose; leukemisch, Hairy-cell leukaemia, hairy cell leukemie, Haarcelleukemie, Hairy-cell leukemia, Harige-cellenleukemie, Leukemie, Harige-cellen-, Leukemische reticulo-endotheliose, Reticulo-endotheliose, leukemische
German Haarzell-Leukaemie, leukaemische Retikuloendotheliose, Haarzellenleukaemie, Haarzellleukaemie, Haarzelleukämie, Leukämie, Haarzell-, Leukämische Retikuloendotheliose, Retikuloendotheliose, leukämische
Italian Reticoloendoteliosi leucemica, Reticuloendoteliosi leucemica, Leucemia a cellule capellute
Portuguese Reticulendoteliose leucémica, LEUCEMIA DE CELULAS CABELUDAS, Tricocitoleucemia, Leucemia de Células Pilosas, Reticuloendoteliose Leucêmica
Spanish Reticuloendoteliosis leucémica, [M]Leukaemic reticuloendotheliosis, [M]Leukemic reticuloendotheliosis, Leucemia de Células Vellosas, Leucemia de Celulas Vellosas, Leucemia de Celulas Peludas, Tricoleucemia, Leucemia de Células Peludas, (M)leucemia de células vellosas, leucemia de células pilosas, leucemia de células vellosas, leucemia de tricoleucocitos (anomalía morfológica), leucemia de tricoleucocitos, reticuloendoteliosis leucémica (clínica), reticuloendoteliosis leucémica (concepto no activo), reticuloendoteliosis leucémica de localizaciones no especificadas (trastorno), reticuloendoteliosis leucémica de localizaciones no especificadas, reticuloendoteliosis leucémica, SAI (trastorno), reticuloendoteliosis leucémica, SAI, reticuloendoteliosis leucémica, tricoleucemia (clínica), tricoleucemia (trastorno), tricoleucemia, Leucemia de células pilosas, Leucemia de Células Pilosas, Reticuloendoteliosis Leucémica, Leucemia de Celulas Pilosas, Reticuloendoteliosis Leucemica
Swedish Hårcellsleukemi
Japanese ﾓｳﾖｳｻｲﾎﾞｳﾊｯｹﾂﾋﾞｮｳ, ﾊｯｹﾂﾋﾞｮｳｾｲｻｲﾓｳﾅｲﾋｼｮｳ, 白血病性細網内皮症, ヘアリー･セル白血病, 白血病-ヘアリーセル, 細網症-白血性, ヘアリーセル白血病, 有毛細胞白血病, 毛様細胞白血病, 毛髪様細胞白血病, 白血性細網内皮症, 白血性細網症, ヘアリー細胞白血病, 白血病-有毛細胞, 白血病-毛様細胞, 細網内皮症-白血性
Czech leukémie s vlasatými buňkami, Vlasatobuněčná leukemie, Leukemická retikuloendotelióza
Finnish Karvasoluleukemia
Russian LEIKOZNYI RETIKULOENDOTELIOZ, RETIKULOENDOTELIOZ LEIKOZNYI, LEIKOZ VOLOSATOKLETOCHNYI, VOLOSATOKLETOCHNYI LEIKOZ, ВОЛОСАТОКЛЕТОЧНЫЙ ЛЕЙКОЗ, ЛЕЙКОЗ ВОЛОСАТОКЛЕТОЧНЫЙ, ЛЕЙКОЗНЫЙ РЕТИКУЛОЭНДОТЕЛИОЗ, РЕТИКУЛОЭНДОТЕЛИОЗ ЛЕЙКОЗНЫЙ
Korean 유모세포 백혈병
Polish Białaczka kosmatokomórkowa, Siatkowico-śródbłonkowica białaczkowa, Białaczka włochatokomórkowa
Hungarian Hajas sejtes leukaemia, Leukemiás reticuloendotheliosis, Leukaemiás reticuloendotheliosis, Hajassejtes leukemia
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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