Abstract
We report a rare case of primary bone marrow lymphoma with cold-type autoimmune hemolytic anemia (AIHA). A 70-year-old Japanese woman with suspected liver disorder presented to our hospital with palpitation. On physical examination, she had jaundice and signs of anemia. No lymphadenopathy or hepatosplenomegaly was noted. A direct antiglobulin test was positive for complement C3b and C3d. Anti-IgG testing was negative. Cold agglutinin was positive with a titer of 1:≥8,192, and haptoglobin was absent. A diagnosis of cold-type AIHA was made. Bone marrow biopsy revealed involvement with a population of lymphocytes that were positive for CD20 (L-26), CD79a, and Bcl-2. No lymphoma lesion was detected on computerized tomography or on upper and lower endoscopy. The patient was diagnosed with diffuse large B cell lymphoma (DLBCL) presenting with cold-type AIHA. She was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone, resulting in complete remission after six cycles. As of 22 months after presentation, no signs of cold-type AIHA or lymphoma were present.
This is a preview of subscription content, log in via an institution to check access.
References
Genry I, Michel M, Hermine O et al (2002) Characteristics of autoimmune hemolytic anemia in adults: retrospective analysis of 83 cases. Rev Med Intern 23:901–909
Berentsen S, Ulvestad E, Langholm R et al (2006) Primary chronic cold agglutinin disease: a population based clinical study of 86 patients. Haematologica 91:460–466
Berentsen S, Beiske K, Tjonnfjold GE (2007) Primary chronic cold agglutinin disease: an update on pathogenesis, clinical features and therapy. Hematology 12:361–370
Berentsen S (2011) How manage cold agglutinin disease. British J Hematol 153:309–317
Sumi M, Ichikawa N, Shimizu I et al (2007) Primary diffuse large B-cell lymphoma of the bone marrow complicated with autoimmune hemolytic anemia and erythroid hypoplasia. Rinsho Ketsueki 48(7):571–575
Ni Áinle F, Hamnvik OP, Glumann C et al (2008) Diffuse large B-cell lymphoma with isolated bone marrow involvement presenting with secondary cold agglutinin disease. Int J Lab Hematol 30(5):444–445
Czuczman MS, Grillo-López AJ, White CA et al (1999) Treatment of patients with low-grade B-cell lymphoma with the combination of chimeric ant-CD20 monoclonal antibody and CHOP chemotherapy. J Clin Oncol 17(1):268–276
Martinez A, Ponzoni M, Agostinelli C et al (2012) Primary bone marrow lymphoma: an uncommon extranodal presentation of aggressive non-Hodgkin lymphomas. Am J Surg Pathol 36(2):296–304
Alvares CL, Matutes E, Scully MA et al (2004) Isolated bone marrow involvement in diffuse large B cell lymphoma: a report of three cases with review of morphological, immunophenotypic and cytogenetic findings. Leuk Lymphoma 45(4):769–775
Jaffe ES, Harris NL, Stein H et al (2008) Introduction and overview of the classification of the lymphoid neoplasms. WHO classification of tumors of haematopoietic and lymphoid tissues. International Agency for Research on Cancer Press, Lyon
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kosugi, S., Watanabe, M. & Hoshikawa, M. Primary Bone Marrow Lymphoma Presenting with Cold-Type Autoimmune Hemolytic Anemia. Indian J Hematol Blood Transfus 30 (Suppl 1), 271–274 (2014). https://doi.org/10.1007/s12288-014-0356-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12288-014-0356-6