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Abstract: PO0323

Paraneoplastic Manifestations of Mantle Cell Lymphoma

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Harry, Chantelle, Morristown Medical Center, Morristown, New Jersey, United States
  • Patel, Shivangi, Morristown Medical Center, Morristown, New Jersey, United States
  • King, Judy Ann, Louisiana State University, Baton Rouge, Louisiana, United States
Introduction

Mantle cell lymphoma (MCL) is a rare and aggressive form of Non Hodgkins Lymphoma. Pathological patterns involving extra nodal sites are either the result of lymphoid infiltration or secondary mechanisms such as immune complex deposition triggering paraneoplastic disease, that can be revealing of the underlying pathology.

Case Description

58-year-old Hispanic male with a history of hypertension, hyperlipidemia presented with nontender, nonpruritic rash from buttocks down that evolved from a petechial scattered rash into a maroon-colored, palpable purpura. He had no associated fever, chills, abdominal symptoms, or weight loss. Labs were notable for WBC 13.3/nL, Hgb 11 g/dL, Platelets 100/nL, Creatinine 2.3mg/dL (Creatinine 1 mg/dL 6/25/2019), AST/ALT 87/70 U/L, ESR 50 mm/hr, CRP 69 mg/L and urinalysis with large blood and 4-10 RBCs, Protein/Creatinine Ratio, Urine 0.34 mg/dL. RUS showed normal size kidneys and 19.5 cm spleen. Patient received 1-gram IV Methylprednisone for presumed IgA nephropathy. Complement levels were low, not consistent with IgA Nephropathy leading to a negative serological workup for infectious disease and secondary glomerulonephritis. Renal biopsy showed immune complex mediated GN with C1q positivity and diffuse foot process effacement. CT scan of abdomen showed splenomegaly with markedly enlarged lymph nodes throughout the abdomen and pelvis. Further outpatient workup was positive for skin biopsy with leukocytoclastic vasculitis. Lymph node biopsy was positive for Ki-67 of 70%, cyclin D1 along with chromosomal analysis positive for t(11:14) confirming mantle cell lymphoma. He was enrolled in a research study which includes Rituxan, bendamustine and high dose cytarabine.

Discussion

Developing a rash led the patient to seek medical attention. Combination of rash, hematuria and abnormal creatinine presumed a diagnosis of IgA nephropathy, however both skin and renal biopsy showed immune complex mediated pathology representing paraneoplastic disease. Further workup revealed splenomegaly and lymphadenopathy (LAD) eventually diagnosing MCL. MCL classically presents with B cell symptoms and LAD, but it is important to recognize that extra-nodal manifestations can unmask and lead to an earlier diagnosis and treatment. Management of MCL also treated his paraneoplastic pathology as seen by improved renal function and no recurrence of rash.