Iranian Journal of Blood and Cancer
Volume:4 Issue: 3, Spring 2012

Sickle cell anemia is relatively common in Khuzestan province located in Southwest Iran. The characteristics of sickle cell disease in Iran are apparently different from other regions; some of these characteristics might be related to β-chain haplotypes. The purpose of this study was to determine the frequency of β-chain haplotypes in 50 patients with homozygous sickle cell anemia in Khuzestan Province. The haplotypes were explored around and within the ε–Gγ–Aγ–ѱβ–δ–β globin gene complex by analysing seven polymorphic restriction sites [(1) HincII 5ʹ to ε (2) XmnI-5ʹγG، (3) Hind III-γG، (4) Hind III-γA، (5) HincII- ѱβ، (6) HincII- 3ʹto Ψβ، and (7) HinfI and RsaI - 5ʹto β]، followed by restriction digestion and agarose gel electrophoresis. The effect of beta globin haplotypes upon hematologic parameters such as hemoglobin، hemoglobin F، mean corpuscular hemoglobin concentration، reticulocyte count، serum bilirubin and Lactate Dehydrogenase was also studied. The Arab/Indian was the most frequent haplotype، present in 38 percent of chromosomes، followed by Benin haplotype (18%)، Senegal haplotype (16%)، Bantu haplotype (16%) and Cameroon haplotype (12%). The mean percentage of hemoglobin F in sickle cell anemia patients was 17. 18±8. 81%، and in the homozygous Arab/Indian haplotype it was higher (20. 90%)، but the difference was not significant. The hemoglobin F was significantly higher in females compared to males (19. 10 versus 14. 50، P<0. 005). There was no significant correlation between haplotypes and hematological characteristics like fetal hemoglobin level among our patients.

To study whether the generated CD1a positive cells belong to the leukemic cells among patients with juvenile myelomonocytic leukemia. We used mononuclear cells from 3 patients with juvenile myelomonocytic leukemia, from which two had monosomy 7. The mononuclear cells from these patients were cultured in RPMI/10%FCS without adding exogeneous growth factors for 7 days. At day 7 the cultured cells were harvested and analyzed using antibodies against Ki 67, CD20 and CD1a. Additionally the cultured cells were analyzed using antibodies against CD1a and CD20 and chromosome 7 specific DNA probe, using combined fluorescence immunophenotyping and interphase cytogenetic techniques. The immunocytochemistry assay demonstrated that a high number of cells were in proliferation status, which was determined by antibody against proliferation associated nuclear protein ki 67. The percentage of Ki 67 positive cells was between 24% and 38% respectively. The percentage of CD1a positive cells was between 8% and 31% and the percentage of CD20 positive cells was between 5% and 12% respectively. The fluorescence immunophenotyping and interphase cytogenetic analysis showed that nearly all CD1a positive cells of one patient with monosomy 7 had one chromosome 7, whereas in other patient with monosomy 7, the amount of CD1a positive cells having only one chromosome 7 was approximately 11%. Furthermore, the combined immunophenotyping and cytogenetic analysis showed that the CD20 positive cells in all patients had normal karyotype. Our results suggest that CD1a positive cells generated by mononuclear cells from patients with juvenile myelomonocytic leukemia in vitro most probably belong to the leukemic cells. Since monosomy 7 could not be detected in all CD1a positive cells in juvenile myelomonocytic leukemia patients with monosomy 7, it is to assume that monosomy 7 is a secondary event in the pathophysiology of juvenile myelomonocytic leukemia.

Previous studies about the relationship between bone marrow megakaryocyte count and the chronicity of ITP has yielded paradoxical results. The aim of the present study was to investigate any relationship between the megakaryocyte count in the bone marrow and the chronicity of ITP. This study was performed to compare the primary bone marrow aspiration megakaryocyte count، obtained early upon the diagnosis of ITP، between chronic ITP (case) and acute ITP (control) groups among patients aged less than 15 years old، at Mofid Hospital، Tehran and Amir-Kabir Hospital، Arak. Data collected was analyzed using SPSS version 18. The project was approved by local Ethics committee and written informed consent was obtained from all parents. Three hundred and seven patients with ITP including 212 patients with acute ITP (69. 1 %) and 95 patients with chronic ITP (30. 9 %) participated in the study. The bone marrow megakaryocyte count was increased in 263 patients (85. 7 %)، decreased in 7 patients (2. 3 %)، and normal in 37 patients (12. 1%). Among 212 patients with acute ITP، the bone marrow megakaryocyte count was increased in 182 patients (85. 8 %)، decreased in 5 patients (2. 4%)، and normal in 25 patients (11. 8 %). Among 95 patients with chronic ITP، the bone marrow megakaryocyte count was increased in 81 patients (85. 3 %)، decreased in 2 patients (2. 1 %)، and normal in 12 patients (12. 6%). There was no statistically significant difference between two groups regarding the megakaryocyte count. Based on our findings it seems that the variant of bone marrow megakaryocyte count is not related to the chronicity among patients with ITP.

Hodgkin’s disease (HD) is a neoplastic disease originating in lymphoid tissue، which spreads to lymphoid structures and ultimately nonlymphoid tissues. Lactate Dehydrogenase and Alkaline Phosphatase are increased in blood following membrane cell damage. The aim of this study was to compare Lactate Dehydrogenase and Alkaline Phosphatase levels in children in different stages of Hodgkin’s Lymphoma with normal children. In the present study، the sera from 30 patients who suffered from Hodgkin’s Lymphoma and were referred to Children’s Medical Center، Tehran، Iran as well as 30 normal subjects were collected. The mean age was 7. 5 years among patients and 6. 8 years in normal subjects. Lactate Dehydrogenase and Alkaline Phosphatase levels were measured using kinetic and colorimetric methods. Data were analyzed using Pearson’s correlation coefficient and t- test. Stages III and IV (advanced stages) were observed in 61. 4% of the patients. Lactate Dehydrogenase level among patients was statistically higher in comparison with controls (P<0. 01). There was also a statistically significant increase in Alkaline Phosphatase level among patients in comparison with controls (P<0. 05). A comparison of both Lactate Dehydrogenase and Alkaline Phosphatase levels among patients with advanced stages (III، IV) and those with initial stages (I، II)، revealed elevated levels among patients in advanced stages (P<0. 001). Lactate Dehydrogenase and Alkaline Phosphatase levels might be utilized as markers to determine Hodgkin’s lymphoma severity in addition to other markers.

Breast cancer is an important cause of cancer death among women all over the world. Even in places with low incidence there are reports of an increase in the number of breast cancer patients. The aim of the present study was to report the demographic aspects of breast cancer among patients referred by surgeons for adjuvant therapy in a private cancer clinic during the past five years in Fars province, Iran. One hundred and two patients were reviewed. The age of the patients and pathologic data including size, site, histopathology of tumor, axillary lymph node involvement, Estrogen receptor and HER2/neu (human epidermal growth factor type2) receptor status were collected. Thirty percent of cases were 40-49 years old which was the most common age group, and the average age was 46 years. Most of the patients had grade II and grade III histopathology, 59 and 36 percent respectively. Modified radical mastectomy was performed in 85% of cases and in 15% of cases a breast conservation surgery was performed. The average age for breast cancer was low, the detection was delayed and the rate of breast conserving surgery was very low among our patients. This indicates a need for more studies to rule out any genetic or acquired factors contributing to this earlier appearance of disease among our population.

Platelet activation and adhesion to the site of vascular injury is a dynamic process comprising reversible and irreversible phases. Platelet adhesion typically occurs in a multi-step process similar to the selectin/integrin-mediated adhesion of neutrophils. This phenomenon is highly regulated and influenced by the cross-talk between platelets and injured endothelium. This cross-talk involves a variety of mediators including adhesion molecules and receptors, agonists, chemokines, shed proteins and various proinflammatory lipids. This review briefly discuses the main adhesion molecules and receptors involved in both reversible and irreversible phases of platelet adhesion to the site of vascular injury, leading to a better characterization of the multistep mechanisms of thrombus formation.

This is a report concerning a concurrent case of hemoglobin J Iran (Hb J Iran) and Hemoglobin H (Hb H) disease in an Iranian woman. The patient was coincidentally found during the course of routine pre-marital genetic counselling for her son. The diagnosis of heterozygote Hb J Iran for her son, ultimately led to the diagnosis of concurrent Hb J Iran and Hb H disease. The hematological examination of the patient showed a microcytic, hypochromic anemia, and hemoglobin electrophoresis on cellulose acetate media at alkaline PH depicted a profile of fast moving hemoglobins consisting of Hb J Iran, Hb Bart and hemoglobin H. Molecular analysis of alpha and beta chains of hemoglobin revealed a genotype of -(α)20.5/-α3.7+ β β77 His -> Asp. To our knowledge, this is the first report of such patient with details of hematological and molecular analysis from south west Iran. This case report may provide a new insight into hemoglobin electrophoresis interpretation and hemoglobin disorders especially for health sector and genetic counsellors.

Literature review shows that hepatitis C infection may increase the risk of non-hodgkin lymphoma. Reactivation of hepatitis B infection has been reported in patients with hairy cell leukemia after chemotherapy and/or splenectomy. We present hepatitis B surface (HBs) antigenemia and concurrent hepatitis C virus (HCV) seropositivity in a case of hairy cell leukemia.