[Lymphoplasmacytic lymphoma accompanied by severe myelofibrosis]

Rinsho Ketsueki. 2023;64(1):54-59. doi: 10.11406/rinketsu.64.54.
[Article in Japanese]


A 61-year-old female was referred to our hospital because of pancytopenia and febrile neutropenia. On admission, computed tomography showed mild hepatosplenomegaly and intra-abdominal abscess formation in the right pelvic region; however, no lymphadenopathy was found. Bone marrow (BM) examination showed severe fibrosis by silver staining. Several small- to medium-sized lymphocytes with a constriction in the nuclei were observed, exhibiting CD3 (-), CD10 (-), CD20 (+), BCL-2 (+-), and CD138 (+-). Genetic testing revealed that BM cells were positive for MYD88 mutation and positive for IgH rearrangement, whereas neither JAK2 nor CALR mutation was positive. A diagnosis of BM infiltration of lymphoplasmacytic lymphoma (LPL) was made. Rituximab monotherapy was administered once a week for four times. BM examination 4 weeks after the end of treatment showed that lymphoma cells had disappeared and that myelofibrosis had been almost gone. The MYD88 mutation of BM turned out to be negative at that moment.

Keywords: Lymphoplasmacytic lymphoma; MYD88 mutation; Myelofibrosis.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Bone Marrow / pathology
  • Female
  • Humans
  • Lymphoma, B-Cell* / diagnosis
  • Middle Aged
  • Myeloid Differentiation Factor 88 / genetics
  • Primary Myelofibrosis* / complications
  • Primary Myelofibrosis* / drug therapy
  • Primary Myelofibrosis* / genetics
  • Rituximab
  • Waldenstrom Macroglobulinemia* / complications
  • Waldenstrom Macroglobulinemia* / drug therapy
  • Waldenstrom Macroglobulinemia* / genetics


  • Myeloid Differentiation Factor 88
  • Rituximab