Triple immunosuppressive therapy can accelerate the recovery of antibody-mediated pure red cell aplasia and allow successful concurrent resumption of erythropoietin.

Antibody (Ab)-mediated pure red cell aplasia (PRCA) is a rare hematologic disorder. For the first time here, the authors report the use of combination therapy which consists of mycophenolate mofetil 500-1000 mg/day, intravenous cyclophosphamide 600 mg monthly and monthly intravenous methylprednisolone 1 gm/day for 2 days followed by oral prednisolone 10 mg/day. A 62-year-old woman developed Ab-mediated PRCA after using subcutaneous erythropoietin-beta 3000 U weekly for 14 months at the predialysis stage. Ab-mediated PRCA was diagnosed based on (1) the transfusion need of more than 1 unit/wk to keep hemoglobin level stable, (2) corrected reticulocyte count 0.36% and (3) < 5% erythroblasts with normal myeloid cells and megakaryocytes in bone marrow biopsy. Serum assay confirmed the anti-erythropoietin antibody of 230 ng/mL. The patient recovered from PRCA after the triple immunosuppressive therapy for 3 months. The rapid recovery occurred despite the fact that the patient was receiving intravenous erythropoietin-alpha while having the antibody in the serum. The present case describes the acceleration of the recovery and successful resumption of erythropoietin concurrently despite the positive serum anti-erythropoietin antibody.

Adjunctive corticosteroid therapy in 149 grade II (non-shock) adult DHF patients: an analysis during January 2008-February 2010.

BACKGROUND: It is well known that immunopathogenesis play an important role in the development of severe complications in DHF. Since 2006, the authors have experience in giving immunomodulators to save life of many severe complicated adult DHF patients. This experience stimulates our interest on the benefit of adjunctive corticosteroid therapy in adult grade II DHF patients. OBJECTIVE: To find out whether there are some benefits of giving adjunctive corticosteroid therapy in adult grade II DHF patients. DESIGN OF THE STUDY: Retrospective analysis during January 2008-February 2010. MATERIAL AND METHOD: One hundred and forty nine adult grade II DHF patients were admitted at Vichaiyut Hospital. They were divided into 3 groups according to the different therapy designed by the responsible clinicians. Group 1 consisted of 59 cases who received full dose-short course of intravenous dexamethasone (4 milligram every 6 hours for 2-3 days). Group 2 consisted of 61 cases who received intermittent 4 milligrams intravenous dexamethasone only at febrile episode and group 3-29 cases did not received corticosteroid. All the patients were investigated similarly. Age, sex, symptoms and signs including daily hematologic studies (Hct, Wbc, differential count, platelet count) were recorded. Serum SGOT SGPT bilirubin, alkaline phosphatase and albumin BUN, creatinine were performed on admission and repeated as indicated. The parameter to measure the benefit of adjunctive corticosteroid included 1) severity of thrombocytopenia, 2) liver impairment, 3) the days of illness as determined by fever and 4) the length of the hospital days. RESULTS: The clinical severity of all the three groups were quite similar. There was no benefit of using adjunctive corticosteroid therapy in term of changing the severity of thrombocytopenia and liver impairment. However, the days of illness and the length of hospital days were shorter at 4.6 days and 3.7 days respectively in the group who received full dose, continuous-short course of dexamethasone intravenously. This is statistically significant when compared to the other two groups who had the longer total days of fever at 5.8 days and 6.03 days and the longer length of hospital days at 5.19 days and 4.5 days respectively (p < 0.05). CONCLUSION: Adjunctive corticosteroid by given full dose, continuous short course in grade II adult DHF reduced the course of illness (days of fever) and the length of hospital days. These findings indicated the benefit of using adjunctive corticosteroid therapy in grade II adult DHF patients.

Hemophagocytic syndrome in Dengue hemorrhagic fever with severe multiorgan complications.

A 46 year old woman who presented with severe multiorgans involvement including liver brain, cardio-pulmonary failure, gastrointestinal bleeding, progressive cytopenia, DIC and hemophagocytic syndrome during the convalescent phase of Dengue type II has been successfully treated primarily with pulse methyl prednisolone and high dose intravenous immunoglobulin G. The authors believe that HPCS are not infrequently seen with high mortality and recommended early diagnosis and treatment with the regimen. This is the first complete report of hemophagocytic syndrome in adult dengue hemorrhagic fever in Thailand. The literature of HPCS in DHF was reviewed and discussed.

EBV and hemophagocytic syndrome: analysis of 3 cases, with speculation on clinical features, therapy and role of EBV.

The authors report three patients diagnosed with EBV associated HPCS. The first case died of a fatal EBV infection. The second and third cases had primary disease of malignant lymphoma. In case 2, T cell lymphoma associated HPCS was diagnosed early. However, despite the aggressive treatment of HPCS and T-cell lymphoma, the patient died because of the refractory lymphoma. In case 3, HPCS and B malignant lymphoma were diagnosed at post mortem. EBV was found very late in all three cases. Case 1 and case 2 had a very high DNA-EBV load in blood. Case 3 demonstrated EBV-RNA encoded antigen (EBER) in lymph node by in situ hybridization technique. The clinical features of HPCS were analysed. Four early manifestations in these three cases were emphasized, namely fever, splenomegaly, progressive pancytopenia and impaired liver function test without severe jaundice. The authors stress the most important factor to save the life of the patients is to give early diagnosis and early proper management of HPCS as well as the etiologic diseases. The treatment of choice of early HPCS are pulse corticosteroid, IVIgG. Combination immunochemotherapy including pulse corsticosteroid, IVIgG, cyclosporin A, etoposide and plasma exchange should be given promptly in severe cases. From the present report, it indicates that the association of EBV with HPCS is not uncommon in Thailand. EBV is very important because it gives a very poor prognosis either by being an etiologic cause of HPCS or by association with ML with HPCS. Clinicians should be aware of EBV and recognize it early. The early treatment of EBV should helpfully changes the prognosis of the patients. The role of EBV on the occurrence of HPCS and T-ML is also discussed