Rare Diseases Symptoms Automatic Extraction

Waldenström macroglobulinemia: 2013 update on diagnosis, risk stratification, and management.

[waldenström macroglobulinemia]

Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy.The presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis.Age, hemoglobin level, platelet count, β2 microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis.Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab-based therapy is used in virtually all US patients with WM and can be combined with alkylating agent or purine nucleoside analog (or both). The preferred Mayo Clinic nonstudy therapeutic induction is rituximab, cyclophosphamide, and dexamethasone. Future stem cell transplantation should be considered in induction therapy selection.Bortezomib, thalidomide, everolimus, lenalidomide, and bendamustine have all been shown to have activity in WM. Given WM's natural history, reduction of complications will be a priority for future treatment trials.

Diseases presenting "hepatosplenomegaly" symptom

  • alpha-thalassemia
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • gm1 gangliosidosis
  • homocystinuria without methylmalonic aciduria
  • neonatal adrenoleukodystrophy
  • oculocutaneous albinism
  • omenn syndrome
  • severe combined immunodeficiency
  • waldenström macroglobulinemia
  • zellweger syndrome

This symptom has already been validated