title: Primary bone marrow diffuse large B-cell lymphoma accompanying cold agglutinin disease: A case report with review of the literature.
abstract: Cold agglutinin disease (CAD) is a well-recognized complication of lymphoproliferative disorders. It has been previously recognized that cases of primary CAD frequently exhibit underlying malignant lymphoma in the bone marrow. Lymphoplasmacytic lymphoma is the most common subtype of malignant lymphoma; however, diffuse large B-cell lymphoma (DLBCL) has also been documented, albeit extremely rare. The current report presents a case of primary bone marrow DLBCL accompanying CAD. A 76-year-old male presented with fever and fatigue. Laboratory tests revealed anemia and elevated bilirubin and cold agglutinins with a titer of 8,192 at 4°C. Bone marrow biopsy demonstrated DLBCL and systemic surveillance failed to detect tumorous lesions or lymphadenopathy. Following R-THP-COP therapy, cold agglutinins titer was markedly decreased (by <4); however, malignant lymphoma relapsed and cold agglutinin levels increased again (4,096). This is the second documented case of primary bone marrow DLBCL accompanying CAD. Previously, malignant lymphoma exclusively involving the bone marrow, namely primary bone marrow lymphoma (PBML), has been recognized as a rare and aggressive subtype. The analyses of the present study revealed that the incidence of hemolytic anemia in primary bone marrow DLBCL may be high compared with conventional DLBCL. Therefore, additional analyses are required to clarify the clinicopathological features of PBML.
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Blood Service Center, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Yamashita
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Blood Service Center, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Moro
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Blood Service Center, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Yumoto
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Blood Service Center, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Uchibayashi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Miyuki
authors.initials: M
authors.lastname: Yoshii
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Ryota
authors.initials: R
authors.lastname: Nakanishi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Okuno
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshida
authors.affiliation: Department of Hematology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Okuno
authors.affiliation: Division of Blood Service Center, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Department of Hematology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hodohara
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Okabe
keywords: cold agglutinin disease; diffuse large B cell lymphoma; primary bone marrow lymphoma