Geographic heterogeneity of cytogenetic characteristics of acute myeloid leukemia in the early detection: a comparative study of Iranian and Indian adult patients

Acute Meyloid Leukemia [AML] in adults is known to be a heterogeneous disease with diverse chromosome abnormalities. Some of these chromosome abnormalities are found with a high incidence in populations from specific geographical areas and ethnic societies. Therefore, we studied the cytogenetic features of AML cases in contrasting societies of Iran and India. Cytogenetic investigation was performed in various subtypes of AML with unstimulated short-term culture and High Resolution Cell Synchronization with some modification. Cytogenetically, Iranian M3 displayed a higher frequency of t[15;17] than Indian M3 [33.8% vs 19.3%] followed by M2 [t[8;21] [27.7% vs 16.2%]] and M1 [t[9;22] [16.0% vs 11.3%]]; whereas, inv[16]11q23 and numerical chromosomal aberrations in chromosome 5,7,8 occurred more frequently in Indian than Iranian. These findings represented different cytogenetic characteristics of t[15;17] between the two populations. This is the first systematic cytogenetic study of an ethnic Iranian population. Extensive biological studies of AML in Iran and India and various countries to be needed to clarify the role of genetic as well as geographic heterogeneity in the pathogenesis of AML

Surveying the efficacy of hypercvad regimen in acute lymphoblastic leukemia patients

Acute lymphoblastic leukemia [ALL] is a malignant disorder with the origin of B or T lymphocyte progenitor. This malignancy presents with anemia, thrombocytopenia and neutropenia due to suppression of hematopoiesis by increasing blast cells in bone marrow. With regard to many reports on effectiveness of HyperCVAD regimen in ALL patients and inadequate data from Iranian patients, the present study was conducted to assess the efficacy of this regimen in Iranian patients. This comparative before-after study included 36 adults with newly diagnosed ALL referred to Taleghani hospital between September 2004 and September 2007. Treatment consisted of four cycles of HyperCVAD alternating with four cycles of high dose methotrexate [MTX] and cytarabine with intrathecal prophylaxis and supportive care with antibiotic prophylaxis and G-CSF. Maintenance included 2 years of treatment with mercaptopurine, MTX, vincristine and prednisolone. Results of treatment on complete remission and survival were evaluated. Mean age of patients was 21 +/- 11.3 years. Mature B-cell disease [Burkitt-type] was present in 2.7%, T-cell disease in 25%, and leukocytosis of more than 30_10[9]/L, Philadelphia chromosome, and mediastinal mass were found in 27.8%, 8%, and 8% of patients, respectively.The complete response and mortality during induction were 91.6% and 2.7%, respectively, while estimated median survival time and estimated median CR duration were 13 and 8.3 months, respectively. Our results revealed that efficacy of HyperCVAD regimen in our patients were inferior to other studies. We recommend another study with larger sample size to compare the efficacy of this regimen with other standard regimens

[Primary plasma cell leukemia: effective treatment with intermediate dose melphalan]

Primary Plasma cell Leukemia [PCL] is a rare disease with no standard treatment, although, combination chemotherapy, BMT and intermediate dose melphalan have been shown to be effective, in some case reports. Patients usually present with anemia, thrombocytopenia, hypercalcemia, and renal failure. Diagnosis is confirmed by peripheral and bone marrow examination. We recently had a case in our department. A 43 years old gentleman presented with history of fatigue, weakness, weight loss, dyspnea and bone pain. Diagnosis of PCL was confirmed by PBS and bone marrow exam. The patient was treated using single intermediate dose melphalan [60 mg/m2/ IV] plus Dexamethasone with G-CSF support; after 3 weeks, complete remission was achieved. In the last visit, done 9 months after treatment, he was doing well clinically and his Lab data were normal. This case report confirmed the efficacy of intermediate dose of melphalan in the management of plasma cell leukemia

Determining the mutation and the first reports of May Hegglin anomaly from Iran and the first homozygous E1841K mutations in the world

May-Hegglin anomaly [MHA] is a rare autosomal disorder which is characterized by triad of thrombocytopenia, giant platelets and Dohle like inclusion bodies in granulocytes. This is the first report of MHA and its mutation from Iran. The specimen of two patients [father 51 and son 15 y/o] collected with EDTA and tri-sodium citrate anticoagulants. CBC and peripheral blood smear studied by automatic cell counter and microscopic examination, respectively. Direct sequencing of extracted DNA of certain exons of MYH9 gene was performed. Both patients had demonstrated the diagnostic triad of MHA. Mutations showed homozygous and heterozygous pattern in the father and the son, respectively. This is the first report of MHA from Iran. The mutation of both patients was E1841K which is the most common type among MYH9 mutations in MHA. The most interesting finding was the homozygous mutation that did not entail any clinical severity