Primary bone marrow diffuse large B cell lymphoma: a case series and review

Abstract

Diffuse large cell lymphoma involving bone marrow is not uncommon, but primary, de novo, bone marrow diffuse large B cell lymphoma (DLBCL) is rare. To understand the clinical features and outcomes of this distinct entity, we collected 12 cases in 5 years from a single-center database. They accounted for 1.16% of lymphoma, or 2.65% of diffuse large B cell lymphoma. Nine cases presented with fever of unknown origin. Lactate dehydrogenase levels were elevated in all but one case. Nine cases belonged to the high-risk group according to their international prognosis indexes (score 4 or 5). Four patients received no chemotherapy, all of whom died within 1 month. Four patients received cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP)-like chemotherapy, and their median survival was 13 months. Finally, four patients received rituximab 375 mg/m(2) in addition to CHOP-like chemotherapy. All of them had complete remission and three are still alive without relapse. We concluded that primary bone marrow DLBCL is a rare but distinctive subtype of lymphoma. The prognosis for this entity is poor but rituximab-based treatment is promising for improving its outcomes.

Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

“…The following criteria were used for diagnosing lymphoid neoplasia in the BM as PBML: pathologically confirmed BM infiltration with NHL, regardless of peripheral blood involvement; no evidence of lymph‐node involvement, defined as enlargement of any lymph‐node to >1 cm on imaging studies (including thoracic, abdominal, and pelvic computerized tomography [CT] scans); the absence of tumor formation; and exclusion of leukemia/lymphoma cases with primarily BM involvement, including chronic lymphocytic leukemia/small lymphocytic lymphoma, prolymphocytic leukemia, lymphoplasmacytic lymphoma, mantle cell lymphoma, splenic marginal zone lymphoma, hairy‐cell leukemia, Burkitt lymphoma, and acute lymphoblastic leukemia 2, 3, 4…”

“…However, several recently reported cases exhibited an uncommon NHL, without lymphadenopathy but only a primary tumor in the BM—primary bone marrow lymphoma (PBML) 1, 2, 3, 4, 5, 6…”

“…The current knowledge regarding PBML has mainly been derived from several clinicopathologic reports,1, 2, 3, 4, 5, 6 and most studies of PBML report two diagnostic criteria in common: pathological evidence of BM infiltration with NHL and the absence of any lymph‐node enlargement or tumor formation 1, 2, 3, 4. However, there are two major limitations to our current understanding of this condition.…”

“…First, the definitions of PBML used in previous studies varied, partly due to its rarity, heterogeneous histology, and a lack of uniform pathological features 1, 2, 3, 4. Second, PBML often initially manifests as prolonged fever or fever of unknown origin (FUO), which is usually first examined by general practitioners 1, 2, 3, 4, 5, 7, 8, 9, 10. Hence, the diagnosis of PBML is often delayed, leading to a poor prognosis and a median overall survival (OS) of <18 months 1, 2, 3, 4.…”

“…Second, PBML often initially manifests as prolonged fever or fever of unknown origin (FUO), which is usually first examined by general practitioners 1, 2, 3, 4, 5, 7, 8, 9, 10. Hence, the diagnosis of PBML is often delayed, leading to a poor prognosis and a median overall survival (OS) of <18 months 1, 2, 3, 4. Therefore, it is essential that hematologists can recognize patients with underlying PBML at an early stage, when they are consulted regarding FUO patients.…”