CAVITARY PRIMARY PULMONARY LYMPHOPLASMOCYTIC LYMPHOMA COMPLICATING HENOCH–SCHÖNLEIN PURPURA

Abstract

Non-Hodgkin lymphoma (NHL) may occur in the chest, often as secondary involvement but occasionally as primary disease. Low-grade pulmonary B-cell lymphoma is the most frequent form. The diagnosis based on histological examination of surgical samples. Henoch–Schönlein purpura (HSP) as a systemic vasculitis typically less commonly affects adults. Triggers including infections, medications and malignancy for HSP have been recognized. We report a patient presenting with HSP who had primary pulmonary lymphoplasmocytic lymphoma (PPLL) as an underlying malignancy. Case: 57-year-old male patient developed chest pain with a hemoglobin level 5.9g/dL. Symptoms resolved after erythrocyte transfusions. He has been diagnosed as having type 2 myocardial infarction. The detailed investigation contributed to warm autoimmune hemolytic anemia (AIHA) diagnosis. Steroid was started. He had high eryhtrocyte sedimentation rate. Further workup revealed bilateral multiple hilar lymphadenopathies and nodular cavitary pulmonary lesions on torax CT. The clinical picture and laboratory evaluation were not consistent with invasive fungal infection and tuberculosis. Purified protein derivative (PPD) skin test was negative. Bronchoalveolar lavage did not reveal any atypical cell and culture positivity. Thoracoscopic lymph node excision was performed. Histologic investigation showed plasma cells in the paracortical area with a slight increase in kappa to lambda ratio (3:1). A fine needle aspiration biopsy of lung tissue revealed lymphoplasmocytosis. PET-CT documented cavitary nodular lesions and hilar lympadenomegalies but no other suspicious lesion. Biopsy sample from one lesion sized 18 × 12 mm with SUVmax 5 revealed plasma cell infiltration with an IgG kappa phenotype. PPLL was diagnosed. Meanwhile AIHA responded to steroid but recurred during dose tapering. PPLL treatment with bortezomib and rituximab based regimen was decided. AIHA went in remission but relapsed after one year with HSP associated clinical picture. He had severe abdominal pain with intestinal wall thickness. Biopsy samples from kidney showed IgA vasculitis and from skin granular type of IgA and C3 deposition in the walls of small diameter vessels in the papillary dermis. Pulse steroid followed by cyclophosphamide controlled the clinical picture. We wished to highlight that in adults presenting with HSP may be a sign of underlying malignancy relapse.