Rare Diseases Symptoms Automatic Extraction

Distinct characteristics and new prognostic scoring system for Chinese patients with Waldenström macroglobulinemia.

[waldenström macroglobulinemia]

Waldenström macroglobulinemia (WM) is an uncommon lymphoid malignancy. The characteristics and prognosis of WM have never been systematically studied in the East.We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients, and compared them with the Western reports.The median age was 62 years old with a male-to-female ratio of 3.74. The most common symptoms at diagnosis were fatigue (77.8%) and bleeding (20%), while only 6 patients (6.7%) were asymptomatic. In the univariate analysis, age >62 years, thrombocytopenia, leucopenia, cytopenias ≥ 2, and high risk on the international prognostic scoring system for WM were the adverse risk factors, but only age >62 years and ≥ 2 cytopenias were the independent prognostic factors in the multivariate analysis. Using age <62 years and ≥ 2 cytopenias, three significantly different prognostic groups could been distinguished, with 5-year overall survival of 71.6%, 48.6%, and 17.0% (P < 0.001).Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.

Diseases presenting "high risk" symptom

  • 22q11.2 deletion syndrome
  • acute rheumatic fever
  • adrenal incidentaloma
  • alpha-thalassemia
  • aniridia
  • canavan disease
  • congenital diaphragmatic hernia
  • congenital toxoplasmosis
  • cowden syndrome
  • cushing syndrome
  • cutaneous mastocytosis
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • esophageal squamous cell carcinoma
  • fabry disease
  • harlequin ichthyosis
  • heparin-induced thrombocytopenia
  • hirschsprung disease
  • hodgkin lymphoma, classical
  • homocystinuria without methylmalonic aciduria
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • krabbe disease
  • legionellosis
  • liposarcoma
  • locked-in syndrome
  • oligodontia
  • oral submucous fibrosis
  • papillon-lefèvre syndrome
  • pendred syndrome
  • phenylketonuria
  • primary hyperoxaluria type 1
  • severe combined immunodeficiency
  • sneddon syndrome
  • waldenström macroglobulinemia
  • werner syndrome

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