Intravascular large B cell lymphoma presenting as anasarca: an unusual presentation of a rare lymphoma

Abstract

Dear Editor, An 82-year-old male presented with progressive generalized edema over a month without associated fever, angina, dyspnea, rash, lymphadenopathy, and neurological or gastrointestinal symptoms. He was found to be profoundly hypotensive requiring vasopressor support. Initial blood work revealed severe hypoalbuminemia of 1.6 g/dL and a markedly elevated LDH of 1891 U/L. Complete blood count revealed normocytic anemia with hemoglobin of 9.8 g/dL and normal platelet and leukocyte count. Peripheral smear showed no abnormal cells. Liver function tests, urinalysis, chest X-ray, and echocardiogram were unremarkable. Random skin biopsies were subsequently performed revealing intravascular infiltration of CD5and CD20-positive malignant cells compatible with intravascular B cell lymphoma (IVLBCL; Fig. 1). IVLBCL is a rare malignancy with estimated incidence of less than one per million per year [1]. It was first described as endothelial cell tumor [2]. Subsequent immunohistochemical studies confirmed its lymphoidal nature [3]. In 2008, WHO classified IVLBCL as a subtype of extranodal diffuse large B cell lymphoma characterized by limited proliferation of lymphoma cell within lumina of small blood vessels [4]. Diagnosis of IVLBCL can be challenging given its bewildering presentation. Virtually, any organ can be affected as a result of small vessel occlusion. Fever, night sweat, and weight loss are the most common presentation. In western countries, these constitutional symptoms are frequently accompanied by neurological and cutaneous symptoms, whereas hemophagocytic syndrome is more predominant in Asian population [1, 5]. Anarsarca is an unusual initial presentation and was found in less than 5 % in one case series [5]. Biopsy is mandatory for the diagnosis. Involved tissues demonstrate large lymphoid cells with prominent nucleoli and frequent mitotic figures in the capillaries and venules. B cell-associated antigens, particularly CD20, CD5, and CD10, are typically positive. Selection of organ for biopsy should be guided by patient's manifestation as identification of tumor cells is relatively easy when an affected organ is biopsied [6]. In case of no apparent specific organ involvement, as seen in our patient, random skin biopsy from normal-appearing skin is an alternative diagnostic approach with a high sensitivity of 83.3 % [7]. Prognosis of a patient with IVLBCL is unfavorable although recent retrospective studies suggest more promising outcome with the use of rituximab in conjunction with anthracycline-based chemotherapy [1, 8]. Our patient, with unstable hemodynamics despite of vigorous resuscitation, was not a suitable candidate for instituting chemotherapy. Unfortunately, his clinical course deteriorated and he passed away after a week of hospitalization.