II. Pathophysiology

  1. Lymphoproliferative Malignancy
    1. Proliferation of plasma cells and Lymphocytes
  2. Similar to Multiple Myeloma
  3. Produces a large monoclonal IgM protein

III. Epidemiology

  1. Older men more frequently affected
  2. Hereditary component

IV. Symptoms

  1. Weakness
  2. Fatigue
  3. Oronasal bleeding
  4. Blurred vision or Decreased Visual Acuity (33%)
  5. No Bone pain
    1. Contrast with Multiple Myeloma
  6. Hyperviscosity results in neurologic symptoms
    1. Dizziness or Vertigo
    2. Headache
    3. Nystagmus
    4. Paresthesias
    5. Decreased Level of Consciousness (Somnolence to Coma)
  7. Sudden Deafness
  8. Progressive spinal muscular atrophy

V. Signs

  1. Pallor
  2. Splenomegaly
  3. Hepatomegaly
  4. Peripheral Lymphadenopathy
  5. Retinal lesions
    1. Retinal Hemorrhage
    2. Retinal Exudate
    3. Venous congestion
  6. Peripheral Neuropathy
  7. Pulmonary Disease
    1. Infiltrates or Pleural Effusion

VI. Labs

  1. Complete Blood Count
    1. Normocytic normochromic Anemia
  2. Peripheral Smear
    1. Rouleau formation
  3. Erythrocyte Sedimentation Rate markedly elevated
  4. Bone Marrow Biopsy
    1. Hypocellular
    2. Extensive Infiltrations by lymphoid and plasma cells
    3. Increased Mast Cell numbers
  5. Immunoelectrophoresis
    1. Monoclonal IgM Protein >3 g/dl

VII. Differential Diagnosis

VIII. Management

  1. Suppression of Lymphocyte and Plasma Cell proliferation
    1. Chlorambucil
    2. Cyclophosphamide
    3. Combination chemotherapeutic regimens
  2. Hyperviscosity
    1. Plasmapheresis with cell separator

IX. Prognosis

  1. Five Year median survival after diagnosis

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Ontology: Waldenstrom Macroglobulinemia (C0024419)

Definition (MSHCZE) Krevní onemocnění charakterizované přítomností monoklonálního makroglobulinu tj. IgM produkovaného patologickou populací plasmatických buněk. Patří k malignitám z plasmatických buněk, podobá se mnohočetnému myelomu (nezpůsobuje poškození kostí) a lymfoplasmocytárnímu lymfomu. Je relativně vzácná, vyskytuje se obv. ve vyšším věku a jejím nápadným příznakem je zejm. hyperviskózní syndrom s poruchami zraku, nervovými příznaky, krvácením z nosu aj. K dalším příznakům patří hepatosplenomegalie, lymfadenopatie, anemie, krvácivé projevy. V léčbě se používají některá cytostatika, symptomatická terapie, plasmaferéza, nověji i ovlivnění markerů (anti-CD20 protilátky) na populaci nádorových buněk. Doba přežití je značně variabilní, může dosahovat přes 10 let. Asymptomatičtí pacienti se neléčí a sledují se. (cit. Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) An indolent (slow-growing) type of non-Hodgkin lymphoma marked by abnormal levels of IgM antibodies in the blood and an enlarged liver, spleen, or lymph nodes.
Definition (NCI) Lymphoplasmacytic lymphoma associated with bone marrow involvement and IgM monoclonal gammopathy.
Definition (CSP) plasma cell dyscrasia resembling leukemia, with cells of lymphocytic, plasmacytic, or intermediate morphology, that secrete an IgM monoclonal component.
Definition (MSH) A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity.
Concepts Neoplastic Process (T191)
MSH D008258
ICD9 273.3
ICD10 C88.0 , M9761/3
SnomedCT 190818004, 35562000, 154750002, 190014001, 109981009, 190821002, 267503004, 190817009
English Macroglobulinemia, Macroglobulinemia, Waldenstrom's, Waldenstrom's Macroglobulinemia, Waldenstroms Macroglobulinemia, Macroglobulinaemia NOS, Waldenstrom's macroglobulinaem, Macroglobulinemia NOS, WALDENSTROMS MACROGLOBULINEMIA, [M]Waldenstrom's macroglobulin, [M]Waldenstrom's macroglobulinaemia, [M]Waldenstrom's macroglobulinemia, Waldenstrom's macroglobulin, Lymphoma, Lymphocytic, Plasmacytoid, Lymphoma, Lymphoplasmacytoid, Lymphomas, Lymphoplasmacytoid, Lymphoplasmacytoid Lymphoma, Macroglobulinemia, Primary, Primary Macroglobulinemia, Lymphoplasmacytoid Lymphomas, Waldenstrom's macroglobulinemia (diagnosis), Macroglobulinemia, Waldenstrom, Von Waldenstrom macroglobulinaemia, Waldenstrom's macroglobulinaemia NOS, Von Waldenstrom macroglobulinemia, Waldenstrom's macroglobulinemia NOS, Waldenstrom Macroglobulinemia, Waldenström macroglobulinemia (disorder), Waldenström macroglobulinemia, Waldenstrom's macroglobulinemia (disorder), Waldenström macroglobulinaemia, Waldenstrom macroglobulinemia, Waldenstrom Macroglobulinemia [Disease/Finding], Waldenstroms Macroglobulinaemia, Waldenstrom's Macroglobulinaemia, Macroglobulinaemia, Waldenstrom's, Waldenstrom Macroglobulinaemia, waldenstroms macroglobulinemia, waldenstrom macroglobulinemia, waldenstrom's macroglobulinaemia, waldenstrom's macroglobulinemia, lymphoplasmacytoid lymphoma, macroglobulinaemia, macroglobulinemia waldenstrom, macroglobulinemia waldenstroms, lymphoplasmacytic lymphoma, macroglobulinemia waldenstrom's, Waldenstrom's macroglobulinaemias, Waldenstrom's macroglob'naemia, Waldenström macroglobulinemia (clinical), Macroglobulinemia NOS (disorder), [M]Waldenstrom's macroglobulinaemia (disorder), Waldenström macroglobulinaemia (clinical), Primary macroglobulinemia, Waldenstrom's macroglobulinemia, Macroglobulinaemia, Waldenstrom's macroglobulinaemia, Primary macroglobulinaemia, Macroglobulinemia (disorder), Waldenstrom's macroglobulinemia (morphologic abnormality), macroglobulinemia, plasmacytoma, macroglobulinemia, macroglobulinemia, Waldenstrom's, Waldenström; macroglobulinemia, macroglobulinemia; Waldenström
Italian Macroglobulinemie di Waldenstrom, Macroglobulinemia di Waldenstrom, NAS, Macroglobulinemia, Macroglobulinemia NAS, Macroglobulinemia di Waldenstrom
Spanish Macroglobulinemia primaria de Waldenström, Macroglobulinemia de Von Waldenström, Macroglobulinemia de Von Waldenstrom, Macroglobulinemia de Waldenstrom NEOM, Macroglobulinemia de Waldenstrom, Macroglobulinemia NEOM, Linfoma Linfoplasmacitoide, macroglobulinemia de Waldenstrom, macroglobulinemia de Waldenstrom (trastorno), macroglobulinemia de Waldenström (trastorno), macroglobulinemia de Waldenström (clínico), macroglobulinemia, SAI, macroglobulinemia, SAI (trastorno), macroglobulinemia de Waldenström (clínico) (trastorno), macroglobulinemia (trastorno), macroglobulinemia de Waldenström (anomalía morfológica), macroglobulinemia de Waldenström, macroglobulinemia primaria, macroglobulinemia, Macroglobulinemia, Macroglobulinemia de Waldenström
Dutch macroglobulinemie, macroglobulinemie NAO, Von Waldenstrom macroglobulinemie, Waldenströms macroglobulinemie NAO, macroglobulinemie van Von Waldenström, Waldenström; macroglobulinemie, macroglobulinemie; Waldenström, Macroglobulinemie van Waldenstrml, Waldenströms macroglobulinemie, Waldenströms macroglobulinemieën, Globulinemie, macro-, Macroglobulinemie, Waldenström-macroglobulinemie
French Macroglobulinémie de Von Waldenstrom, Macroglobulinémie de Waldenstrom SAI, Macroglobulinémie SAI, Macroglobulinémie de Waldenstrom, Macroglobulinémie de Waldenström SAI, Lymphome lympho-plasmacytoïde, Lymphome lymphoplasmacytoïde, MGW (MacroGlobulinémie de Waldenström), Macroglobulinémies de Waldenström, Maladie de Waldenström, Macroglobulinémie, Macroglobulinémie de Waldenström
German Makroglobulinaemie, Makroglobulinaemie NNB, Waldenstroem Makroglobulinaemie NNB, Waldenstrom-Makroglobulinaemie NNB, Von Waldenstrom-Makroglobulinaemie, Waldenstrom-Makroglobulinaemie, Makroglobulinaemie Waldenstroem, Waldenstroem Makroglobulinaemien, Waldenstroem Makroglobulinaemie, Makroglobulinämie, Waldenström-Makroglobulinämie
Portuguese Macroglobulinemia de von Waldenström, Macroglobulinemia de Waldenström NE, Macroglobulinemia NE, Linfoma Linfoplasmacitoide, Macroglobulinemias de Waldenstrom, Macroglobulinemia de Waldenstrom, Macroglobulinemia, Macroglobulinemia de Waldenström
Japanese マクログロブリン血症NOS, フォン・ワルデンストローム・マクログロブリン血症, ワルデンストローム・マクログロブリン血症NOS, ワルデンストローム・マクログロブリン血症, マクログロブリンケッショウNOS, ワルデンストロームマクログロブリンケッショウNOS, マクログロブリンケッショウ, フォンワルデンストロームマクログロブリンケッショウ, ワルデンストロームマクログロブリンケツショウNOS, マクログロブリンケツショウ, フォンワルデンストロームマクログロブリンケツショウ, ワルデンストロームマクログロブリンケツショウ, ワルデンストロームマクログロブリンケッショウ, マクログロブリンケツショウNOS, ワルデンシュトレーム・マクログロブリネミア, ワルデンシュトレーム・マクログロブリン血症, ワルデンシュトローム・マクログロブリネミア, ワルデンシュトローム・マクログロブリン血症, 形質球リンパ球性リンパ腫, マクログロブリン血症, リンパ腫-形質球リンパ球性, Waldenstroemマクログロブリン血症, Waldenstromマクログロブリン血症, Waldenstroemマクログロブリンネミア, Waldenstromマクログロブリンネミア, マクログロブリネミア, ワルデンシュトレーム症候群, ワルデンシュトローム症候群
Swedish Waldenströms makroglobulinemi
Czech Waldenströmova makroglobulinémie, Von Waldenströmova makroglobulinemie, Waldenströmova choroba, Makroglobulinemie, Makroglobulinemie NOS, Waldenströmova makroglobulinemie NOS, Waldenströmovy makroglobulinemie, primární makroglobulinémie, lymfoplazmocytoidní lymfom, Waldenströmova makroglobulinemie
Finnish Waldenströmin makroglobulinemia
Russian MAKROGLOBULINEMIIA, VAL'DENSTREMA MAKROGLOBULINEMIIA, ВАЛЬДЕНСТРЕМА МАКРОГЛОБУЛИНЕМИЯ, МАКРОГЛОБУЛИНЕМИЯ
Korean 왈덴스트룀 마크로글로불린혈증
Polish Makroglobulinemia, Choroba Waldenstroma, Makroglobulinemia Waldenstroma
Hungarian Von Waldenström macroglobulinemia, Waldenström-macroglobulinaemia k.m.n., Macroglobulinemia, Von Waldenström macroglobulinaemia, Waldenström macroglobulinaemia, Macroglobulinaemia, Waldenström-macroglobulinaemia, Macroglobulinaemia k.m.n., Waldenström macroglobulinemia, Waldenström macroglobulinaemia k.m.n.
Norwegian Waldenströms makroglobulinemi, Waldenstrøms sykdom, Waldenströms sykdom, Waldenströms syndrom, Waldenstrøms Makroglobulinemi, Makroglobulinemi