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Intravascular large B-cell lymphoma: masquerade as membranoproliferative glomerulonephritis Mahesha Vankalakunti1, B. Vasavi1 and Aleya Anitha2 1
Department of Nephropathology, Manipal Hospital, Bangalore, India; and 2Department of Nephrology, Kavery Hospital, Hosur, India
Correspondence: Mahesha Vankalakunti, Department of Pathology and Laboratory Medicine, Manipal Hospital, #98, Rustom Bagh, Bangalore, Karnataka, India 560017. E-mail:
[email protected] Kidney International (2016) 90, 231–232; http://dx.doi.org/10.1016/j.kint.2016.03.018 Elsevier Inc. All rights reserved.
Copyright ª 2016, International Society of Nephrology. Published by
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55-year-old female with hypertension for 3 years presented with proteinuria (1.7 g/d). Her physical examination was unremarkable except for conjunctival pallor. Laboratory test revealed anemia (hemoglobin 6.7 gm/dl), with normal leukocytes and platelets. Peripheral smear showed mild lymphocytosis without any abnormal cells. Erythrocyte sedimentation rate was 110 mm Hg/h. Urinalysis showed 3þ albumin, and no RBC or WBC. Serum creatinine was 0.8 mg/dl, and serum albumin was 3.8 gm/dl. Serum electrophoresis was normal. Serum lactate dehydrogenase was elevated, 1660 U/l (normal 160–290 U/l). Abdominal ultrasound showed 9.9 and 10.2 cm kidney size with increased echoes, and mild hepatosplenomegaly. Renal biopsy showed diffuse, global engorged capillaries with medium-large monotonousappearing lymphoid cells possessing stippled chromatin, single to triple nucleoli, and moderate cytoplasm, with brisk mitotic activity (Figure 1). Segmental splitting of capillary basement membranes was noticed. Immunofluorescence was negative. Immunohistochemically, lymphoid cells were positive for CD20 and BcL-2 and negative for CD3/CD68 markers. Ki-67 was >80% (Figure 2). Computed tomography of the chest, abdomen, and pelvis did not reveal any lymphadenopathy or mass lesions. A diagnosis of intravascular large B-cell lymphoma was made. Bone marrow was unremarkable, without any evidence of hemophagocytosis or lymphoma infiltration. The patient was initiated on cyclophosphamide, adriamycin, vincristine, and prednisone. Intravascular large B-cell lymphoma, as the name specifies, is proliferation of clonal lymphocytes that are restricted to vascular lumina, especially small capillaries. Lymphoma cells lack CD29 (b1-integrin) and CD54 (ICAM-1), which are critical for lymphocyte transvascular migration. Intravascular large B-cell lymphoma usually affects elderly patients
Kidney International (2016) 90, 231–232
Figure 1 | Lymphomatous infiltration of glomerular capillaries. Top: original magnification X40, periodic acid–Schiff; bottom: X40, periodic acid–Schiff methanamine silver.
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M Vankalakunti et al.: Intravascular B-cell lymphoma
Figure 2 | Lymphoid cells positive for CD20 and BcL-2 and negative for CD3; Ki-67 was >80%. Original magnification, X40.
with poor performance status, elevated lactic dehydrogenase serum levels, anemia, and B symptoms. This form of lymphoma is rare and underdiagnosed because it is limited
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to the vascular system, typically involving the kidneys, central nervous system, and skin. Glomerular involvement is very uncommon.
Kidney International (2016) 90, 231–232