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 "high frequency" symptom

alpha-thalassemia

canavan disease

classical phenylketonuria

cohen syndrome

cowden syndrome

cystinuria

erdheim-chester disease

familial hypocalciuric hypercalcemia

familial mediterranean fever

gm1 gangliosidosis

heparin-induced thrombocytopenia

hirschsprung disease

homocystinuria without methylmalonic aciduria

inclusion body myositis

kabuki syndrome

kallmann syndrome

krabbe disease

lymphangioleiomyomatosis

neonatal adrenoleukodystrophy

papillon-lefèvre syndrome

pyruvate dehydrogenase deficiency

waldenström macroglobulinemia

x-linked adrenoleukodystrophy

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