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 MYD 88 L 265 P 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 MYD 88 L 265 P mutation . MYD 88 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 MYD 88 mutated LPL /WM had progressive disease , whereas no patient in the MYD 88 unmutated group changed their baseline status . We confirm the high frequency of MYD 88 mutations in LPL /WM . Although the number of MYD 88 wild-type cases was limited , our data indicate that MYD 88 may represent an adverse prognostic marker for LPL /WM .

Diseases
Validation

Diseases presenting "tumor cells" symptom

alpha-thalassemia

carcinoma of the gallbladder

cholangiocarcinoma

cushing syndrome

dedifferentiated liposarcoma

dentin dysplasia

esophageal adenocarcinoma

esophageal carcinoma

esophageal squamous cell carcinoma

hodgkin lymphoma, classical

junctional epidermolysis bullosa

kindler syndrome

liposarcoma

lymphangioleiomyomatosis

pleomorphic liposarcoma

primary effusion lymphoma

severe combined immunodeficiency

triple a syndrome

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

werner syndrome

wiskott-aldrich syndrome

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