II. Epidemiology

  1. Affects patients over age 40 years
  2. More common in Males

III. Pathophysiology

  1. B Lymphocyte proliferation
  2. Commonly associated effects
    1. Splenomegaly
    2. Impaired host defense and secondary infection
    3. Vasculitis

IV. Symptoms

  1. Bone pain (e.g. Hip Pain) if bone lesions present

V. Signs

  1. Defining features
    1. Splenomegaly (75%)
  2. Vasculitis associated findings
    1. Erythema Nodosum
    2. Cutaneous Nodules
  3. Rare findings
    1. Lymphadenopathy
    2. Hepatomegaly

VI. 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

VIII. 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

IX. 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

X. Prevention

XI. Prognosis

  1. Eight year survival >50%

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Ontology: Hairy Cell Leukemia (C0023443)

Definition (MSHCZE) Maligní hematologické onemocnění charakteristické výskytem atypických buněk v krvi, které mají jemné cytoplasmatické vláskovité výběžky. Obvykle se považují za atypické B lymfocyty, mají i některé vlastnosti makrofágů. Probíhá obvykle chronicky s pancytopenií a splenomegalií, s výskytem infekčních komplikací. Léčí se chemoterapií (kladribin) interferonem či splenektomií. (Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) 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 (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 (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, 188653005, 190066005, 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, 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, [M]Hairy cell leukaemia, [M]Hairy cell leukemia, LRE-Leuk reticuloendotheliosis, [M]Leukaem reticuloendothelsis, Leukaem reticuloendothelsis, [M]Leukaemic reticuloendotheliosis, [M]Leukemic reticuloendotheliosis, leukemia hairy-cell, hairy cell leukemia (diagnosis), hairy cell leukemia, Leukaemic reticuloendotheliosis -RETIRED-, hairy-cell leukemia, Leukemia, Hairy Cell [Disease/Finding], hairy cell leukaemia, hairy-cell leukaemia, leukaemia hairy cell, leukemia hairy cell, cell hairy leukemia, cells hairy leukemia, Hairy cell leukemia NOS, Leukemic reticuloendotheliosis NOS (disorder), Leukemic reticuloendotheliosis (morphologic abnormality), Leukemic reticuloendotheliosis of unspecified sites (disorder), [M]Leukaemic reticuloendotheliosis (disorder), Leukemia, Hairy cell, Hairy cell leukemia, Leukemic reticuloendotheliosis, HCL - Hairy cell leukaemia, HCL - Hairy cell leukemia, Hairy cell leukaemia, Leukaemic reticuloendotheliosis, 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, Leucemia de Células Vellosas, reticuloendoteliosis leucémica de localizaciones no especificadas (trastorno), reticuloendoteliosis leucémica (concepto no activo), reticuloendoteliosis leucémica, SAI (trastorno), reticuloendoteliosis leucémica de localizaciones no especificadas, reticuloendoteliosis leucémica, SAI, 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, tricoleucemia (clínica), tricoleucemia (trastorno), tricoleucemia, Leucemia de células pilosas, Leucemia de Células Pilosas, Reticuloendoteliosis Leucémica
Swedish Hårcellsleukemi
Japanese モウヨウサイボウハッケツビョウ, ハッケツビ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
Finnish Karvasoluleukemia
Russian LEIKOZNYI RETIKULOENDOTELIOZ, RETIKULOENDOTELIOZ LEIKOZNYI, LEIKOZ VOLOSATOKLETOCHNYI, VOLOSATOKLETOCHNYI LEIKOZ, ВОЛОСАТОКЛЕТОЧНЫЙ ЛЕЙКОЗ, ЛЕЙКОЗ ВОЛОСАТОКЛЕТОЧНЫЙ, ЛЕЙКОЗНЫЙ РЕТИКУЛОЭНДОТЕЛИОЗ, РЕТИКУЛОЭНДОТЕЛИОЗ ЛЕЙКОЗНЫЙ
Czech Vlasatobuněčná leukemie, Leukemická retikuloendotelióza, leukémie s vlasatými buňkami, leukémie z vlasatých buněk, leukemie z vláskových buněk, leukemická retikuloendotelióza, vlasatobuněčná leukemie, trichocelulární leukemie
Korean 유모세포 백혈병
Polish Białaczka kosmatokomórkowa, Siatkowico-śródbłonkowica białaczkowa, Białaczka włochatokomórkowa
Hungarian Hajas sejtes leukaemia, Leukemiás reticuloendotheliosis, Leukaemiás reticuloendotheliosis, Hajassejtes leukemia
Norwegian Hårcelleleukemi