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Dear Editor, Primary bone marrow lymphoma (PBML) is a rare disease, of which diffuse large B-cell lymphoma (DLBCL) is the most common pathological subtype [1-3]. While T-cell lymphomas are often associated with hemophagocytic lymphohistiocytosis (HLH), non-Hodgkin B-cell lymphomas are rarely observed in patients with HLH [4]. Moreover, fibrillar projections are an uncommon feature of DLBCL [5, 6]. Herein, we report an extremely rare case of malignancy-associated HLH in which the underlying disease was a primary bone marrow (PBM) DLBCL with fibrillar projections. A 57-yr-old man was admitted with a history of fever and 5-kg weight loss occurring over 1-month period. His physical examination was negative for both lymphadenopathy and a notably palpable spleen. Laboratory results indicated bicytopenia (leukocytes 4.8×10/L, hemoglobin 65 g/L, and platelets 68×10/L) and increased levels of ferritin (2,575 μg/L, reference interval [RI]: 20–320 μg/L), soluble CD25 (9,670 U/mL, RI: 124–466 U/ mL), lactate dehydrogenase (LD; 309 IU/L, RI: 120–250 IU/L), and β2-microglobulin (5.0 mg/L, RI: 1.0–2.4 mg/L). Chest, neck, and abdominal-pelvic computational tomography showed mild splenomegaly, but were negative for other organomegaly. Fluorodeoxyglucose whole-body positron emission tomography revealed hypermetabolic bone marrow (BM) with expansion. BM aspirate smears showed cells (65.4% of nucleated cells) with mediumto large-sized, round nuclei with one or more distinct nucleoli, and a moderate amount of basophilic cytoplasm. These cells had fibrillar projections and occurred in clusters; hemophagocytic histiocytes were frequently observed. A trephine biopsy showed nodular or interstitial aggregations of neoplastic cells with occasional scattered cells in small vessels and no evidence of trabecular bone destruction (Fig. 1). Karyotyping revealed hyperdiploidy and complex abnormalities; 53,XY,add(1) (p36.1),+3,add(7)(q22),+8,del(8)(p21)x2,+9,+17,der(17)del(17) (p13)add(17)(q24)x2,+18,+18,+mar[12]/45,X,-Y[4]/46,XY[4]. Flow cytometry analysis showed neoplastic cells that were CD19+, CD20+, CD22+, FMC7+, CD5+, CD2-, CD3-, CD7-, CD56-, CD10-, CD34-, and TdT-. Immunohistochemistry indicated positivity for CD20, CD5, IRF4/MUM-1, and BCL-6 and negativity for CD10, CD30, BCL-2, and cyclin D1. The Ki67 index was high (90%). In-situ hybridization for the Epstein-Barr virus did not identify infected cells. PCR assay was positive for the clonal rearrangement of the IGH gene (Fig. 2). The patient’s diagnosis was HLH associated with PBML, which

A Case of Primary Bone Marrow Diffuse Large B-cell Lymphoma Presenting With Fibrillar Projections and Hemophagocytic Lymphohistiocytosis