Rare Diseases Symptoms Automatic Extraction

MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart.

[waldenström macroglobulinemia]

In a first series from India, we report 32 cases of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) over 7 years. Here, we analyzed 32 patients with LPL/WM for MYD88 L265P mutation and correlated mutation staus with hematological and biochemical parameters and also with the International Prognostic Scoring System (ISSWM) and treatment response. Twenty-seven out of 32 cases of LPL/WM (84.3%) harbored the MYD88 L265P mutation. MYD88 wild-type WM was associated with a lower number of tumor cells (p < 0.01) and older age (p = 0.02) and a lower ISSWM score at presentation (p = 0.03) as compared to mutated LPL/WM. On evaluation of response (n = 23), 44.4% of patients with MYD88 mutated LPL/WM had progressive disease, whereas no patient in the MYD88 unmutated group changed their baseline status. We confirm the high frequency of MYD88 mutations in LPL/WM. Although the number of MYD88 wild-type cases was limited, our data indicate that MYD88 may represent an adverse prognostic marker for LPL/WM.

Diseases presenting "progressive disease" symptom

  • adrenomyeloneuropathy
  • allergic bronchopulmonary aspergillosis
  • benign recurrent intrahepatic cholestasis
  • cadasil
  • cutaneous mastocytosis
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • inclusion body myositis
  • lymphangioleiomyomatosis
  • pleomorphic liposarcoma
  • severe combined immunodeficiency
  • waldenström macroglobulinemia

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