A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab.

Current treatment options of essential mixed cryoglobulinemia (EMC); include immunosuppressive approaches, such as corticosteroids, cyclophosphamide, plasma exchange, other cytotoxic drugs in moderate to severe manifestations. Some controlled studies have been carried out to assess the efficacy of anti-CD20 monoclonal antibody, rituximab in patients with hepatitis C (HCV) related cryoglobulinemia (CG) and in patients with autoimmune disorders. Recent trials and some case reports demonstrate a beneficial role for rituximab in HCV related mixed CG. Although, the published evidence for treatment of EMC with rituximab is restricted to case reports, which have shown positive results. Several diseases include lymphoproliferative and myeloproliferative disorders, solid tumors, immunological disorders, cardiovascular disorders and some drugs associated with acquired von Willebrand disease (avWD). CG, which is a kind of immune complex disease, may be related with development of autoantibodies to various autoantigens. In this present case report, we showed the efficacy of rituximab in a 21-year-old female patient, suffered from neuropathy and arthralgia related with EMC, and developed avWD, presented with mucosal bleeding associated with CG. von Willebrand factor activity of our patient also increased with controlling the underlying disease, EMC by rituximab. This case demonstrate that rituximab may be an effective treatment option in EMC and avWD mainly related to CG.

Factor V Leiden mutation in venous thrombosis in southeast Turkey.

Venous thrombosis (VT) is a common disease, with an annual incidence in the general population of approximately 1 per 1,000. Factor V Leiden mutation (G1691A) (FVL) is the most common risk factor in venous thrombosis. The prevalence of FVL for thrombosis varies greatly in different regions of the world. FVL mutation has been identified both by conventional method and fluorescence resonance energy transfer (FRET) with the LightCycler. Sixty-one patients with VT, different in age and sex, were consecutively entered into this study to assess the prevalence of FVL in VT in southeast Turkey. FVL mutation was found in 24.6% (15/61). Fourteen individuals were heterozygous and 1 homozygous, a rate of 22.9% and 1.6%, respectively. In conclusion, the authors suggest that FVL mutation is common in patients with venous thrombosis in southeast Turkey.

Prothrombin G20210A gene mutation with LightCycler polymerase chain reaction in venous thrombosis and healthy population in the southeast of Turkey.

Venous thrombosis (VT) is a common disease, with an annual incidence in the general population of approximately 1 per 1000. The prevalence of genetic risk factors for thrombosis varies greatly in different parts of the world. Prothrombin G20210A (PT G20210A) gene mutation has been recently identified as a common risk factor in venous thrombosis. Sixty-one patients with VT, differing in age and sex, and 340 healthy subjects were consecutively enrolled into our study to determine the prevalence of PT G20210A in VT and in the healthy population of the southeast of Turkey. The mutation was identified with fluorescence resonance energy transfer (FRET) with the LightCycler polymerase chain reaction. The PT G20210A mutation was found to be 6.5% (4/61) in the VT group and 1.2% (4/340) in the healthy group ( P = 0.021). Three patients with VT had a heterozygous PT G20210A mutation, and the other patient with VT had both Factor V Leiden and PT G20210A mutations. We showed that this method may be used safely for detection of the PT G20210A gene mutation, and the prevalence of PT G20210A mutation is significantly higher in patients with VT than in the healthy population in the southeast of Turkey.

Non-Hodgkin's lymphoma in southeast Turkey: clinicopathologic features of 490 cases.

We have carried out a retrospective analysis of 490 non-Hodgkin's lymphomas (NHLs), followed at our clinic, with the purpose of evaluating the clinicopathologic features of these patients. The patients were assessed with regard to their characteristics including age, gender, histologic distribution, stage, extranodal involvement, presenting symptoms, and biopsied site. Of the patients 314 (64%) were male and 176 (36%) were female. The overall median age was 43 years (range: 14-90). The patients were classified according to the Working Formulation (WF) system: 71 (14.4%) were low grade, 342 (69.8%) were intermediate grade, 43 (8.7%) were high grade, and 34 (6.7%) had other lymphomas. Intermediate-grade non-Hodgkin's lymphomas formed the largest group, of whom 320 patients' paraffin blocks were available for Revised European and American lymphoma (REAL) classification: 78% were B-cell lymphomas, whereas 16% were T/NK lymphomas. Six percent of cases were unclassified lymphomas. Diffuse large B-cell lymphoma (DLBCL) was the most commonly observed histopathologic type in 132 (41%) patients. Extranodal involvement was found in 218 (44.5%) patients. The most commonly affected extranodal sites were small bowel, stomach, and tonsil in 72 (33%), 63 (29%), and 19 (8.7%) patients, respectively. According to the Ann Arbor staging system, the vast majority of patients (89.4%) were advanced stage. In conclusion,The characteristics of NHLs in our region show some differences from the other sites of Turkey and the world.

Hepatitis C virus in patients with non-Hodgkin's lymphoma in southeastern Anatolia region of Turkey: a prospective case-control study of 119 patients.

Hepatitis C virus (HCV) has been associated with several extrahepatic disorders including mixed cryoglobulinemia (MC), autoimmune thyroiditis, Sjogren's syndrome. Such associations have led to the suggestion that HCV may participate in the development of various immunmediated disorders. Recently, it has been hypothesised that HCV might act as a trigger for the development of monoclonal B-cell disorders such as non-Hodgkin's lymphoma (NHL). Discordant data have been reported in different geographic regions of the world. The aim of this prospective case-control study was to detect the prevalence of HCV in patients with NHL in southeastern Anatolia region of Turkey. In this study, HCV antibody prevalence and cryoglobulinemia were investigated in 119 patients with histologically diagnosed NHL. The control group consisted of 117 patients who visited the outpatient clinic of internal medicine. None of the patients had HCV antibody positive (0%) with the enzyme immunoassay and reverse transcriptase polymerase chain reaction (RT-PCR). One of the control patients had positive HCV antibody (0.9%). Our data does not support the association between HCV infection and NHL in southeastern Anatolia region of Turkey.

Treatment of blastic phase chronic myeloid leukemia with mitoxantrone, cytosine arabinoside and high dose methylprednisolone.

Fourteen patients with blastic phase chronic myelogenous leukemia received combination chemotherapy with mitoxantrone 5 mg/m2 intravenously daily for 3 days, cytosine arabinoside 100 mg/m2 intravenously over 2 hours bid for 7 days and high dose methylprednisolone 1000 mg/day intravenously for 5 days. The patients' mean age was 52 +/- 10 (range 34-64) and Philadelphia chromosome was positive in all. Five patients (35%) achieved complete remission and four patients (28%) had a partial remission. Overall remission rate was 64%. The mean survival was 11.1 +/- 8.6 months (median 13) for all patients, 19.4 +/- 4.0 months (median 19) for those achieving a complete remission, 12.50 +/- 5.7 months (median 14) for patients with partial remission and 1.8 +/- 1.8 months (median 2) for the unresponsive patients. Two of 5 unresponsive patients died early after the second course of remission induction. The treatment regimen was generally well tolerated. Marrow hypoplasia was observed in 9 (64%) patients and 7 (50%) had febrile episodes. Non-myelosupressive toxicity of the regimen was acceptable. Nausea and vomiting were observed in 8 (57%) patients and 3 (21%) patients developed flushing due to cytosine arabinoside. These results suggest that the regimen with mitoxantrone, cytosine arabinoside and high dose methylprednisolone in remission-induction of blastic phase chronic myelogenous leukemia may be a valid option that may also improve overall prognosis.

Hodgkin's disease variant of Richter's transformation: a case report.

Hodgkin's disease rarely develops in patients with B-chronic lymphocytic leukemia. Patients developing Hodgkin's disease after the diagnosis of chronic lymphocytic leukemia have been called the "Hodgkin's disease variant of Richter's transformation." We present a 62-yr-old man with a 17-mo history of chronic lymphocytic leukemia, who clinically and hematologically on remission was admitted to our clinic because of rapidly developing right cervical lymphadenopathy. He was diagnosed with lymph node biopsy as a mixed-cellularity Hodgkin's disease.

Ogilvie's syndrome in a patient with multiple myeloma.

Ogilvie's syndrome is characterized by physical examination and radiologic findings indicative of mechanical obstruction but in which no physical obstructive process can be found. Many factors have been associated with this syndrome which include electrolyte imbalance, systemic infection, drugs, and occasionally, neurologic disease. A case of acute colonic pseudoobstruction is presented which developed in a patient with multiple myeloma. The patient presented with severe thoracic pain, persistent and increasing abdominal distention and lack of bowel sounds. Plain radiography and ultrasonography revealed massive dilatation of the right and transverse colon. Nasogastric aspiration was initiated and all analgesic drugs were withdrawn. Erythromycin was given for nine days as prokinetic and a rectal tube was inserted for one day. Abdominal distention gradually disappeared within one day of nasogastric and rectal tube insertion and with multiple myeloma management. Ogilvie's syndrome is a very rare complication of multiple myeloma. Only one case of Ogilvie's syndrome with multiple myeloma has been reported in the literature. This case report of Ogilvie's syndrome in a patient with multiple myeloma is the second case report in the literature.