MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart.
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.
Hematopathology Laboratory, Tata Memorial Centre , Mumbai , India.
Nikhil
N
Patkar
P G
PG
Subramanian
Prashant
P
Deshpande
Kiran
K
Ghodke
Prashant
P
Tembhare
Russel
R
Mascarenhas
Aditi
A
Muranjan
Shruti
S
Chaudhary
Bhausaheb
B
Bagal
Sumeet
S
Gujral
Manju
M
Sengar
Hari
H
Menon
D000368:Aged; D001483:Base Sequence; D014408:Biomarkers, Tumor / Q000235:genetics; D004252:DNA Mutational Analysis; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D020125:Mutation, Missense; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics*; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*
D014408:Biomarkers, Tumor; D053594:Myeloid Differentiation Factor 88
ISSWM; India; Lymphoplasmacytic lymphoma; MYD88 mutation; Waldenström; macroglobulinemia