Comparative expression profile of orphan receptor tyrosine kinase ROR1 in Iranian patients with lymphoid and myeloid leukemias

It has recently been shown that ROR1, a member of the receptor tyrosine kinase family, is overexpressed in leukemic B cells of Chronic Lymphocytic Leukemia [CLL] and a subset of Acute Lymphoblastic Leukemia [ALL]. In this comparative study the expression profile of ROR1 mRNA was investigated in Iranian patients with CLL and Acute Myelogenous Leukemia [AML] and the results were compared with those previously reported in our Iranian ALL patients. RT-PCR was performed on bone marrow and/or peripheral blood samples of 84 CLL and 12 AML patients. CLL samples were classified into immunoglobulin heavy chain variable region [IGHV] gene mutated [n=55] and unmutated [n=29] and also indolent [n=42] and progressive [n=39] subtypes. ROR1 expression was identified in 94% of our CLL patients, but none of the AML patients expressed ROR1. No significant differences were observed between different CLL subtypes for ROR1 expression. Taken together the present data and our previous results on ROR1 expression in ALL, our findings propose ROR1 as a tumor-associated antigen overexpressed in a large proportion of lymphoid [CLL and ALL], but not myeloid [AML] leukemias. Expression of ROR1 seems to be associated to lineage and differentiation stages of leukemic cells with a potential implication for immunotherapy

Bone mineral density in beta thalassemia major and intermedia, correlation with biochemical and hormonal profiles

Expansion of bone marrow cavity and decreased cortical and trabecular bone tissues and osteoporosis are resulted from beta-thalassemia. The aim of this study was to assess bone mineral density [BMD] in patients with beta thalassemia major and intermedia, and to determine their biochemical and hormonal profiles that may affect BMD. In a cross sectional study from October 2004 to April 2006, 305 patients [273 thalassemia major [137 males and 136 females] and 32 thalassemia intermedia [13 males and 19 females]] were evaluated for BMD. Dual x-ray absorptiometry was performed at 3 sites: spine [L2-L4], femoral neck, and radius. Z score< -2.5 was considered as osteoporosis, and between -1 and -2.5 as osteopenia. Z-scores were calculated according to bone density values based on age and sex. Patients were grouped according to age 3-6, 6-10, 10-13, 13-16, and over 16 years old. The stage of puberty was determined according to Tanner staging and its progression was followed. Biochemical and hormonal profiles of patients were recorded. In thalassemia major, mean age was 14 +/- 6.5 years, and mean BMD Z-score of spine, radius and hip were -2.3 +/- 0.9, -2.8 +/- 1.2, and -1.9 +/- 1.4, respectively. Mean age of patients with thalassemia intermedia was 13.4 +/- 6.2 years, and the mean Z-score of spine, radius, and hip were -2.1 +/- 0.9, -2.0 +/- 1.3, and -2.3 +/- 1.3, respectively. Hypogonadism was detected in 36% of thalassemia major and 35% of thalassemia intermedia; but hypothyroidism, diabetes mellitus, and hypoparathyroidism were detected only in thalassemia major with frequency of 2.8%, 1.8%, and 1.2%, respectively. BMD in spine and radius were significantly lower in patients with hypogonadism than in patients with normal puberty [P=0.039 and P=0.015, respectively]. Height Standard Deviation Score [HSDS] was not significantly different in groups of osteoporosis and normal. Osteoporosis was seen in all age groups, and was more common in males than females at spine and radius bones [P<0.001]. It was less common in patients with hypothyroidism, hypoparathyroidism, and diabetes mellitus. BMD Z-Score had significant correlation with serum ferritin only in radius area [P=0.04], and it had no significant correlation with serum Ca, P, Mg and Zn. Our results showed that the patients with thalassemia major and intermedia had low BMD. Patients with hypogonadism and males had lower BMD. Young children also had low bone mass, so early attention is essential

Immunophenotypic subtyping of leukemic cells from Iranian patients with acute lymphoblastic leukaemia: association to disease outcome

Immunophenotypic characterization of the leukemic cells has been widely used as a tool for diagnosis, classification, stratification and prognosis of leukaemia. To investigate the immunophenotypic subtype profiles of Iranian patients with acute lymphoblastic leukemia [ALL] and its association to disease outcome. In this study, a total of 60 Iranian patients with ALL were immunophenotyped by flow cytometry using a panel of monoclonal antibodies specific for CD2, CD3, CD5, CD10, CD13, CD14, CD19, CD20, CD33, CD34, CD45, HLA-DR and TdT molecules. The samples were initially categorized into T-ALL [n=9], B-ALL [n=50] and mixed lineage [n=1] based on the expression patterns of CD3 and CD19 molecules. B-ALL patients could further be classified into four subtypes, including Pro-B [n=7, 11.7%], Pre-B I [n=28, 46.7%], Pre-B II [n=13, 21.7%] and immature/mature B cells [n=2, 3.3%] on the basis of expression of CD10, CD19, CD20, HLA-DR and TdT. Clinical manifestations and laboratory findings of the patients did not reveal association with immunophenotypic sub-types of ALL, with the exception of mediastinal mass and WBC count at the time of diagnosis which were found to be significantly higher in patients with T-ALL compared with B-ALL [p=0.001 and 0.014], respectively. Our results indicate that overall the immunophenotypic profile of Iranian ALL patients is similar to previous reports and it might be used for monitoring of minimal residual disease and prognosis

[Early Manifestation of Ghosal-Type Hemato-Diaphyseal]

Ghosal-type hemato-diaphyseal dysplasia is a rare autosomal recessive disorder with distinctive diaphyseal and metaphyseal dysplasia of long bones and steroid-dependant anemia. The authors describe a 20-month-old girl who had had a severe transfusion-dependent anemia since late infancy and marked locomotion difficulties as a toddler. The diagnosis was established by X-ray bone survey. The anemia was treated with oral prednisolone. Since then, the patient has been doing well on steroid-maintenance therapy and has no more walking difficulties. The incidence of hemato-diaphyseal dysplasia in the Indian subcontinent and Middle East is notable