Multiple Myeloma Presenting as MPO-ANCA Associated Microscopic Polyangiitis

Reports on the association of multiple myeloma with systemic vasculitis are rare and concern, vasculitis due to crystalglobulin deposition in vessels or to polyarteritis nodosa PAN-like type necrotizing vasculitis. The association of multiple myeloma with MPO-ANCA positive microscopic polyangiitis has not yet been described. We report a case of a 72 year old man, who was referred to our clinic for evaluation of recent onset renal failure (serum creatinine 8mg/dl), proteinuria 2g/day, severe anemia (hematocrit 16%) and fever accompanied by malaise, anorexia, weigh loss, myalgia and arthralgia. These symptoms appeared 20 days prior to his admission. There was no evidence of any infection. The renal biopsy showed necrotizing crescentic glomerulonephritis. The MPO-ANCA were positive. Bone marrow biopsy revealed infiltration with IgG-lambda neoplastic plasma cells. The patient was treated with hemodialysis, plasma exchange, corticosteroids and cyclophosphamide. Even though his clinical situation and renal function improved gradually, two months after his admission, he developed acute respiratory insufficiency accompanied by heavy hemoptysis, patchy infiltration on chest X-rays, due to lung vasculitis and died. Multiple myeloma should be considered in the differential diagnosis of hematological neoplasms capable of causing paraneoplastic ANCA positive microscopic polyangiitis

Primary gastrointestinal non-Hodgkin's lymphoma: A clinicopathologic study of 128 cases in Greece. A Hellenic Cooperative Oncology Group study (HeCOG)

The aim of this retrospective study was to illustrate the clinicopathologic data and the treatment results in patients with primary gastrointestinal tract non-Hodgkin's lymphoma (GI NHL). Among 810 patients with NHL, 128 cases (15.8%) were diagnosed as primary GI tract NHL. There were 79 males and 49 females with median age of 62 years. The most common primary site was the stomach (68%). Overall, 67.2% of the patients were in stages I-II, and 32.8% in stages III-IV. Simultaneous involvement of the GI tract and other extranodal sites was observed in 26 patients (20%). Extranodal marginal zone B-cell lymphoma (MZBL) (i.e., low-grade lymphoma of mucosa-associated lymphoid tissue type) accounted for 48.4% of lymphomas. Aggressive lymphomas (diffuse large B-cell lymphoma [DLBL]) accounted for 44.5%. Eighty-three patients (67.5%) achieved complete response (CR), either by surgery (43/ 43 patients, 17 with DLBL and 25 with MZBL) or by primary chemotherapy (40/64 patients, 22 with DLBL and 17 with MZBL). Sixty-two patients remain in CR; 33/43 after surgical resection (13/17 with DLBL and 20/25 patients with MZBL), and 29/40 after only chemotherapy (18/22 with DLBL and 10/17 with MZBL). The major prognostic factor for outcome in the present study was the stage of the disease. Patients with localized lymphoma (stage I and II) had significantly longer DFS and OS (DFS and OS at 3-year: 83% and 87%, respectively) than patients with extended disease (stage III and IV) (DFS and OS at 3-year: 46% and 60%, respectively) (P < 0.0001). The International Prognostic Index (IPI) for patients with aggressive lymphomas was prognostic only for DFS (79% for low-risk patients [IPI score 0-1] vs 49% for higher risk groups [IPI score 0-1] at 3-year, P = 0.0131)

Multifocal extranodal non-Hodgkin lymphoma: A clinicopathologic study of 37 cases in Greece, a Hellenic Cooperative Oncology Group Study

The purpose of this retrospective study was to illustrate the clinicopathological features of patients presenting with multifocal extranodal non-Hodgkin lymphoma (NHL). Among 810 patients with NHL, 37 cases (4.2%) were found to have multiple extranodal involvement (two or more sites). There were 24 men and 13 women, with a median age of 63 years. The majority of these cases (n = 26) had gastric or intestinal (GI) involvement with or without other extranodal sites. Lung along with another extranodal site was relatively common in the present series. Stratification of the 37 cases according to the International Prognostic Index (IPI) showed that 89% of the patients belonged to the high-risk groups. Diffuse large-B -cell lymphoma (DLBCL) accounted for 62%, and mucosa-associated lymphoma tissue (MALT) lymphoma accounted for 27% of all cases. After induction treatment with anthracycline-based regimens, complete remission was achieved in 21 patients (57%), partial remission was achieved in six patients (16%), and seven patients (19%) had no response, while three patients (8%) were nonevaluable. In conclusion, multifocal extranodal NHL is a heterogeneous group of diseases. The majority of them arise at various sites in the GI tract. DLBCL was the most frequent histological subtype followed by MALT lymphoma. Risk group, as defined by the IPI, was predictive of survival. ©AlphaMed Press