Catch the Calcium: T-Cell Histiocyte-Rich B-Cell Lymphoma Presenting as Hypercalcemia

Abstract

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL) that typically presents in middle-aged patients and carries a poor prognosis. Hypercalcemia presenting as the initial manifestation of the disease is rare, with only one other case reported in the literature. We report a case of a 90-year-old male who presented with progressive lethargy and unintentional weight loss. Initial workup showed elevated serum calcium of 14.6 mg/dL, corrected for albumin, and creatinine of 1.51 mg/dL. He had a suppressed iPTH of 6.3 pg/mL and normal PTHrP (13 pg/mL). Computed tomography (CT) scan of the abdomen and pelvis was performed to rule out underlying malignancy, which showed splenomegaly and enlarged retrocrural and porta hepatis lymph nodes. Bone marrow biopsy was performed to evaluate for hematological malignancy, which revealed findings diagnostic of THRLBCL. While rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) is one of the mainstay therapies for DLBCL and has been shown to have comparable outcomes in THRLBCL, there are documented concerns with its toxicity profile limiting the ability of older patients (60 years and older) to complete therapy. Our patient was treated with R-mini-CHOP, which is much better tolerated in this patient demographic. R-mini-CHOP features decreased doses of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with the conventional dose of rituximab. This case discusses a rare subtype of non-Hodgkin lymphoma presenting with a unique manifestation of hypercalcemia. We highlight the importance of thorough investigation for causes of hypercalcemia as well as the efficacy and tolerability of R-mini-CHOP in this elderly patient demographic.

Keywords: Diffuse large B-cell lymphoma; Hypercalcemia; Malignancy; R-mini-CHOP; T-cell/histiocyte-rich large B-cell lymphoma.

Figure 1

Bone marrow biopsy with involvement by large B-cell lymphoma. (a) Marrow core biopsy with nodular involvement by lymphoma (right lower corner) and hypercellular for age marrow with trilineage hematopoiesis (left upper corner) (H&E stain, × 100). (b). Lymphoma infiltrate composed predominantly of small lymphoid cells and occasional histiocytes intermixed with scattered large (arrowhead) lymphoid cells (H&E, × 400); Inset: The large cells have irregular nuclei with prominent nucleoli and are surrounded by small lymphocytes (H&E, × 1,000). (c, d) Large lymphoma cells express B-cell markers OCT-2 and CD79a (arrowhead, × 400). (e) Immunohistochemical stain for CD3 highlights numerous small lymphocytes in the lymphoma infiltrate. There is focal rosette formation (arrowhead) of small CD3⁺ T lymphocytes around the scattered large lymphoma cells (× 400). H&E: hematoxylin and eosin; OCT-2: octamer transcription factor-2.