Iranian Journal of Blood and Cancer
Volume:2 Issue: 3, Spring 2010

Gelatinase-B named MMP-9 (matrix metalloproteinase-9), is a protease that degrades collagen type ІV and V of extracellular matrix. MMP-9 production is increased in various types of cancers including leukemia and has an essential role in tumor invasion, metastasis and angiogenesis. In this study, patterns of MMP-9 activity in a number of leukemic cells have been evaluated in-vitro. Human leukemic T cells (Jurkat and Molt-4) and monocyte (U937) were cultured in complete RPMI-1640 medium. Then the cells were seeded at a density of 10 6 cells/ml and were incubated with different concentrations of PMA (1-25ng/ml) or PHA (1-10 μg/ml) for 24 hours. Afterwards, MMP-9 activity and MMP-9 protein level in cell-conditioned media were evaluated by gelatin zymography and ECL Western blotting, respectively. PHA/ PMA significantly induced MMP-9 activity in Molt-4 and Jurkat cells after 24-hour incubation in a dose-dependent manner compared with untreated control cells. Moreover, PHA/ PMA extensively and dose-dependently augmented MMP-9 activity in U937 cells after 24-hour incubation time compared with untreated control cells. Besides, PHA/ PMA increased MMP-9 level in U937 cells after 24-hour incubation time as was detected by western blotting compared with untreated control cells. According to the results of this study, human leukemic Jurkat, Molt-4, and U937 cells could exhibit MMP-9 activity with different extents. Among these cell lines, it seems that Molt-4 and U937 human leukemia cell lines, which greatly show MMP-9 activity after stimulation with PHA or PMA, may provide valuable tools for screening MMP enhancers or inhibitors and for assessment of regulatory mechanisms of MMP activity.

Pediatric non-Hodgkin lymphoma (NHL) is a diverse collection of diseases, and results from malignant proliferation of lymphoid cells and immune system. NHL involves throughout the body, but bone and primary central nervous system (CNS) lymphomas are its rare presentations. The incidence of NHL in childhood differs according to age and geographic area, but overall constitutes 8–10% of all malignancies in children between 5–19 years of age. The preferred pathologic and molecular biology classification for NHL is based on currently recognized histologic (morphologic), immunophenotypic, and genetic features, and their clinical presentation and course. The clinical manifestations of NHL in children depend on pathological subtype and primary sites of disease. Abdomen and mediastinum are the most frequent primary sites of involvement. Most centers use st. Jude staging system and diagnostic workup. Most patients present with advanced stage and systemic disease. According to pathophysiology of childhood NHL, treatment strategy is based on extent of dissemination and requires attention to emergent complications. Modern treatments have caused dramatic improvement in childhood NHL. We need well conducted international studies in all parts of the world to increase our knowledge to acieve better outcome and prevent late effects in children.

Glanzmann’s Thrombasthenia (GT) is a rare autosomal, recessive, bleeding syndrome. The main aim of this study was to investigate the relationship between symptoms, bleeding severity, and gender and subtypes of GT by platelet immunophenotyping.Ninety five patients with Glanzmann’s Thrombasthenia (GT) were assessed for the expression of GPIIb-IIIa on the platelet surface using flow cytometry, to determine the most common GT subtypes among Iranian patients. We also evaluated the severity of bleeding phenotype, and classified them as mild, moderate, or severe bleeders. On the basis of their platelet GPIIb-IIIa levels, 73 patients (77%) were classified as type I, 16 patients (17%) as type II, and 6 patients (6%) as type III. Historically, 15 of 95 patients had experienced minor bleeding, 32 reported clinically significant bleeding, and 48 patients had suffered severe bleeding. Thirty eight patients had needed packed red blood cell transfusion. However, no significant correlation was found between bleeding severity and subtypes of GT (p >0.05). Our study showed that there was no correlation between quantitative changes in the surface expression of platelet membrane glycoproteins, and the intensity and frequency of bleeding episodes in patients with GT.

During recent years and paralleling the advances in the treatment of patients requiring chemotherapy or long-term total parenteral nutrition (TPN), it has been necessary to provide a chronic central venous access with a low complication rate and long-term availability (months or even years).In our country, this procedure is performed and its technique is refined, but its advantages and complications have not been analyzed and reported.The records of 120 patients who had undergone TIVAD placement in Mofid children’s hospital, Tehran from 1999 to 2005 were retrospectively reviewed. Outcomes and compliance of parents and therapeutic team were evaluated. There were 120 patients, 68 boys (56.6%) and 52 girls (43.3%); with the age range of 3 months to 13 years old. The following postoperative complications were encountered; withdrawal occlusion in 4 patients (3.3%), intraluminal fibrin sheath in one patient (0.8%), severe neutropenia in 3 patients (2.4%), complete intraluminal occlusion of the catheter in one patient (0.8%), fever and chills in 2 patients (1.6%), and catheter dislodgement in only one patient (0.8%). All parents and members of the therapeutic team were pleased with the TIVAD (100% acceptance).TIVAD placement can be performed in infants and children of all ages. In cases where a chronic venous access is needed, the use of this device is appropriate, because of its low complication rate and long-term applicability.

Restless legs syndrome (RLS) is an annoying sensation and an important cause of sleep disturbances in major thalassemia.In a cross-sectional study in 58 major thalassemia patients aged 3 to 25 years in Tabriz children hospital, restless legs syndrome prevalence was evaluated. RLS is a common problem in major thalassemia with prevalence of 24%. It included the most common reason of sleep disorder in thalassemia patients (83%). Restless legs syndrome; is not related to ferritin or iron level and age. Although RLS is more common in females, it is not statistically significant. RLS is a common disorder in thalassemia patients but usually unnoticed by physicians and nursing.