Acquired pure red cell aplasia associated with malignant lymphomas: a nationwide cohort study in Japan for the PRCA Collaborative Study Group.
Pure red cell aplasia (PRCA) has been reported in association with lymphoma as one of the autoimmune diseases seen during the course of lymphoid malignancies. However, the relation of PRCA with the underlying lymphomas remains unclear. The aim of this study was to clarify the histologic subtypes of lymphomas, the chronological sequence of anemia and lymphoma, and the response to treatment. We conducted a nationwide survey in Japan. From a cohort of 185 PRCA patients, 8 patients with lymphoma were evaluated. Histologic subtypes varied and the lymphoma was of the B-cell type in four cases and of the T-cell type in four. Four patients simultaneously developed PRCA and lymphoma. Three patients developed PRCA following lymphoma, two of whom developed anemia during remission of lymphoma. PRCA preceded lymphoma in one patient. Effective chemotherapy was associated with remission of anemia in concurrent lymphoma and PRCA. Overall, anemia responded to chemotherapy and/or immunosuppressive therapy in seven patients. In four responding patients, PRCA remained in durable remission without maintenance immunosuppressive therapy, which is different from a recurrent feature of idiopathic PRCA. We suggest that the mechanism of lymphoma-associated PRCA is heterogeneous and that durable maintenance-free remission of anemia can be obtained in some patients.
Hirokawa
Makoto
M
Division of Hematology and Oncology, Department of Medicine, Akita University School of Medicine, Akita, Japan. mhirokawa@hos.akita-u.ac.jp
Sawada
Ken-ichi
K
Fujishima
Naohito
N
Kawano
Fumio
F
Kimura
Akiro
A
Watanabe
Takashi
T
Arai
Ayako
A
Matsui
Toshimitsu
T
Nakao
Shinji
S
Urabe
Akio
A
Omine
Mitsuhiro
M
Ozawa
Keiya
K
D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D008223:Lymphoma / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D007166:Immunosuppressive Agents