B35-44 Bleeding and Breathless: A Rare Case of Antineutrophil Cytoplasmic Antibody (ANCA) Vasculitis Secondary to B Cell Lymphoma Presenting as Diffuse Alveolar Hemorrhage

Introduction Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis can be idiopathic or associated with underlying diseases. Here is a case of recurrent hemoptysis due to ANCA positivity, ultimately found to have B-cell lymphoma with plasmacytic differentiation. Case 85-year-old male with 80 pack years smoking history, diastolic heart failure, chronic obstructive pulmonary disease (COPD), atrial fibrillation (AF) on anticoagulation and chronic kidney disease (CKD) with recurrent hospitalizations for acute hypoxic respiratory failure presented with dyspnea and hemoptysis. Recurrent admissions were thought to be multifactorial: pneumonia, COPD, heart failure exacerbation. No active bleeding, bleeding diathesis, rash, recurrent sinusitis, oral ulcers or ear infection. Labs significant for low iron level of 18 mcg/dL, elevated inflammatory markers ESR >140 mm/Hr, CRP 53.6 mg/L, beta 2 microglobulin 5.1 mg/L, M spike of 1.01g/dl on SPEP and IgM level 1507 mg/dl. Bone marrow aspiration on hold due to the patient’s tenuous respiratory status. CT of chest showed persistent interlobular septal thickening, ground glass opacifications, emphysematous changes and enlarged lymph nodes. Rheumatologic labs were notable for positive perinuclear-ANCA, titer of 1:1280 and elevated myeloperoxidase Ab of 131 AU/ml. Rheumatoid factor, anti-cyclic-citrullinated peptide antibody, antinuclear antibody and glomerular basement membrane were negative. He required high-flow and was started on pulse-dose steroids: methylprednisolone 500 mg IV q6 for 6 doses, transitioned to 40 mg q6 for 6 doses. Steroids were then tapered accordingly and transitioned to oral. The total duration of the steroids were approximately 30 days. His oxygen requirements improved and was weaned to nasal cannula. Eventually, a bone marrow biopsy was performed, remarkable for IgM kappa monoclonal gammopathy with lymphoplasmacytic lymphoma. He was found to have high disease burden [80% of bone marrow]. The patient was started on rituximab therapy. The plan included weekly infusions of four doses. After receiving two infusions in the hospital, he showed significant improvement and was discharged with plans to complete therapy outpatient. Discussion B cells play a central role in ANCA-associated vasculitis through autoantibody production and neutrophil activation forming extracellular traps leading to endothelial damage. Since there is major involvement of B cells, treatment targeting them with rituximab [monoclonal antibody against CD20-positive cells], has become a cornerstone in management. There have been documented cases of ANCA-associated vasculitis (AAV) occurring in patients with hematologic malignancies, but as a presenting feature is rare. He had no prior rheumatologic condition that could account for these labs hence, lymphoma served as an autoimmune trigger for his vasculitis.