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M Haghshenas

Publications and source records attributed to M Haghshenas.

18 recordsLinked to original sources
PubMed2005

Bone marrow transplantation in thalassemia major patients using "short" anti-thymocyte globulin therapy in Shiraz, Southern Iran.

Allogeneic bone marrow transplantation (BMT) was performed on 113 Iranian transfusion-dependent thalassemia major patients from May 1993 through September 2003. To have at least 2 years follow-up, we report BMT on 90 patients transplanted up to December 2001. The donors were human leukocyte antigen (HLA)-identical, mixed lymphocyte culture (MLC)-nonreactive siblings (n = 74) on parents (n = 6); HLA-identical MLC-reactive siblings (n = 5) or parents (n = 1); and one HLA antigen-mismatched sibling (n = 4). The induction regimen in 11 patients was oral busulfan (BU) (14 mg/kg) and IV cyclophosphamide (CY; 200 mg/kg); in fifteen patients it was BU (15 mg/kg) and cyclophosphamide (CY; 200 mg/kg); in 47 patients, BU (15 mg/kg), CY (200 mg/kg), and short course of anti-thymocyte globulin (ATG, horse; 40 mg/kg including 10 mg/kg on days -2, -1, +1, +2); and in 15 patients, BU (15 mg/kg) CY (200 mg/kg), and ATG (60 to 100 mg/kg; 10 mg/kg at 3 to 5 days before and after BMT). Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and prednisolone. The group who received BU (14 mg/kg) and CY (200 mg/kg), as compared to the group receiving BU (15 mg/kg) and CY (200 mg/kg), was of younger age and lower risk; median age 7 versus 10 years, and 46% versus 7% in Lucarelli's risk group class I (the best prognostic group), respectively. These patients showed a lower disease-free survival (DFS), namely 64% versus 73%, with a follow up of 2 to 10.5 years. Thus from 9.5 years ago, our standard protocol for BU has been 15 mg/kg. The group who received "short" ATG (40 mg/kg), BU (15 mg/kg), and CY (200 mg/kg) showed almost the same outcome as the group who received a higher dose of ATG (60 to 100 mg/kg), namely DFS 72% versus 73%, respectively, despite the fact that half of both groups were included in the Lucarelli's risk group class III (the worst prognostic group) 49% versus 53%. We showed the same DFS for the patients who received BU (15 mg/kg), CY (200 mg/kg), and no ATG compared with the ATG group (73% vs 72%), but 27% of the group without ATG developed grade IV acute GVHD and 54% developed chronic GVHD. In the group with short ATG, 15% and 17% of patients developed grade IV acute and chronic GVHD, respectively. There was no significant difference for falls in platelets and white blood cell or engraftment days and the number of packed red blood cell transfusions among the groups. The median hospital stay was longer for the group with BU (15 mg/kg), CY (200 mg/kg) namely 81 versus 61 to 65 days. Second bone marrow infusions were needed in 6% and 20% of patients who received ATG doses of (40 versus 60 to 100 mg/kg; respectively (1 to 2 month post-BMT). BU at a dose of 15 mg/kg was more effective than 14 mg/kg BU for its myeloablative properties. By adding "short" ATG course to the conditioning regimen, the incidence of grade IV acute and chronic GVHD was reduced in thalassemic patients, especially when an HLA disparity was present.

Adolescent↗
PubMed2002

Bone marrow transplantation for hematological disorders--Shiraz experience.

OBJECTIVE: In the past 8 years, 120 cases of hematological disorders were transplanted from the HLA identical donors. METHOD: Using chemotherapy based conditioning regimen with cyclophosphamide 200 mg/kg and busulfan 15-16 mg/kg, 80 cases of beta-thalassemia major and 35 cases of leukemia and five patients with aplastic anemia had received bone marrow transplantation. RESULT: The five-year-survival in thalassemic group was 72%, for leukemic group (acute and chronic) was 58%, and also for aplastic anemia 65%. Transplantation related mortality was the cause of death in 29 cases. The two major causes of death were acute graft versus host disease and poor medical condition of patients before marrow transplantation. CONCLUSION: At the present time, allogenic marrow transplantation is curative mode of treatment for many hematological diseases.

Adult↗
PubMed1999

Acute graft-versus-host disease in thalassaemic marrow transplantation with low-dose antithymocyte globulin.

Our unit performed transplantations on 21 classes II and III thalassaemic patients (class II patients had either hepatomegaly or portal fibrosis and class III patients had both). We used busulfan (15 mg/kg) and cyclophosphamide (200 mg/kg). Graft-versus-host disease (GVHD) prophylaxis was cyclosporin, prednisolone and low-dose antithymocyte globulin. Our patient data showed a low incidence of acute GVHD following transplantation. We offer this regimen as an acceptable therapy for thalassaemic patients undergoing allogeneic marrow transplantation as a safe clinical procedure, irrespective of the class of patient.

Acute Disease↗
PubMed1998

Visual failure caused by suprasellar extramedullary hematopoiesis in beta thalassemia: case report.

OBJECTIVE AND IMPORTANCE: Small deposits of extramedullary hematopoiesis, acting as epileptogenic foci, have been observed near convexity dura and adjacent to falx cerebri. These foci could potentially grow and act as space-occupying lesions, producing focal neurological deficits. This condition was observed in a unique case of thalassemia major, with progressive visual loss caused by extramedullary hematopoiesis originating from the base of the cranium and extending up to the inferior third ventricle. The patient had been operated on 4 years earlier to relieve the compressive effects of ectopic marrow in the thoracolumbar epidural space. CLINICAL PRESENTATION: A 21-year-old man is reported with progressive visual loss caused by compressive optic neuropathy as a result of extramedullary hematopoiesis. Computed tomography of the head revealed a suprasellar and parasellar enhancing mass originating from the pre-sphenoid cranial base and approaching the lower third ventricle. INTERVENTION: Partial resection of the tumor was accomplished by a transsphenoidal approach. Monthly blood transfusions and low-dose radiotherapy of the appropriate anatomic structures were then performed. The patient's visual acuity improved only modestly. CONCLUSION: Extramedullary hematopoiesis, although extremely rare, could arise from the base of the cranium and act as a parasellar tumor. When any patient with thalassemia major requiring multiple transfusions develops visual failure, appropriate studies should be performed to rule out compression of visual pathways by ectopic marrow, especially if the patient is receiving deferoxamine.

Adult↗
PubMed1993

Beta-thalassemia in southwestern Iran.

Seventeen unrelated beta-thalassemia patients or carriers from Southwestern Iran were examined for the beta-globin gene mutations by polymerase chain reaction amplification of the beta-globin gene and direct genomic sequencing, or by the method of allele-specific oligonucleotide hybridization. Their clinical and hematological characteristics were also recorded. Of 26 potential thalassemia-causing chromosomes examined, 10 different mutations were found. The IVS-II-1 (G-->A) mutation was the most frequent (31%) followed by the IVS-I-6 (T-->C) mutation (15%). Eight mutations were initially described in Mediterranean populations and two were of Kurdish origin. Four of these mutations, both initially described in the Mediterranean region, are reported here for the first time in Iranians. The unexpectedly high number of different mutations that account for beta-thalassemia in this region of Iran suggest migration of chromosomes from distant places and genetic admixture.

Base Sequence↗
PubMed1978

T and B lymphocytes in alpha-chain disease.

The patients studied were diagnosed as suffering from alpha-chain disease by their clinicopathological features, malabsorption findings, X-ray, and presence of abnormal alpha-chain protein in their serum. The objective of the study was to determine any possible defect of the immune system in such patients. The rosette technique and surface immunofluorescence were used to enumerate the circulating T and B lymphocytes in these patients. They were also skin-tested with tuberculin and given sensitizing doses of dinitrochlorobenzene. Their serum immunoglobulins were also quantitated. It was found that the proportion of circulating B lymphocytes was much higher than normal, whereas that of T lymphocytes was lower than normal. Furthermore, they could not be sensitized to DNCB and their skin test to tuberculin was negative. It was concluded that the disease was a B-cell disease of IgA type, associated with low level of cellular immunity.

B-Lymphocytes↗
PubMed1976

Alpha-chain disease and its association with intestinal lymphoma.

Twenty-seven intestinal lymphoma patients were studied. Abnormal alpha-chain immunoglobulin was detected in the sera of seven of these patients. The alpha-chain disease patients were from the rural areas of Southern Iran. They were of low socio-economic status and their age ranged from 15-44 years. Predominant clinical features were malabsorption, diarrhoea , abdominal pain, vomiting, and weight loss. Infiltration of mucosa of the small intestine with plasma cells and also distortion and flattening of the villi were common histopathologic characteristics of these patients. Involvement of mesenteric lymph nodes with infiltration of tumour cells was observed in a number of cases. Protein studies revealed no significant differences between the serum immunoglobulin levels of these patients and normal values. Immunoelectrophoresis using monospecific antiserum against H-chain of human IgA demonstrated the abnormal precipitin band of alpha-chain disease protein.

Adolescent↗
PubMed1974

Cyclic neutropenia.

Explore the source record for details and available documents.

Adrenal Cortex Hormones↗
PubMed1970

Primary lymphoma of the upper small intestine.

Seven patients with primary lymphoma involving the upper small intestine and presenting with diarrhoea, non-specific abdominal pain, and clubbing are reported. The disease appears to be more prevalent in young women, and clinical and radiological findings can provide an excellent preliminary diagnosis which is usually confirmed by peroral biopsy of the small intestine. This type of lymphoma is found to be clinically distinguishable both from the primary intestinal lymphomas reported from western countries and also from gastrointestinal involvement as part of a more systemic disease. It appears to be prevalent in the Middle East, and because of clear clinical, radiological, and histological features, it can be singled out from other primary intestinal lymphomas and considered as a distinct clinical entity.

Abdomen, Acute↗
PubMedDate not supplied

Bone marrow transplantation in thalassaemia patients in Shiraz, Islamic Republic of Iran.

We report the results of allogeneic bone marrow transplantation in 26 female and 37 male patients with beta-thalassaemia major (age range: 2-17 years), performed at Namazi Hospital over the period 1992-99. In all cases, standard conditioning and immunosuppressive regimens were employed. Currently, 50 patients remain thalassaemia-free, 9 of whom have developed chronic graft-versus-host disease. There were 8 deaths among the 13 unsuccessful transplant cases: 4 due to acute uncontrollable graft-versus-host disease, and 4 to non-transplant-related causes such as hypoglycaemia, hypersensitivity reactions and advanced disease. We conclude that allogeneic bone marrow transplantation is an effective therapy for the treatment of beta-thalassaemia major, particularly for patients classified as classes I and II.

Acute Disease↗