Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocyte enzyme deficiency in the world. The epidemiological, biochemical and molecular studies on G6PD enzyme deficiency performed over the past 50 years are summarized herein, with special emphasis on the findings of studies related to the enzyme deficiency in Turkey.

Malaria parasitemia was assessed in 37 known sickle cell anemia patients attending the routine hematological clinic of the University of Benin Teaching Hospital, Benin-City, Edo State. Parasitemia was determined using the quantitative buffy coat analysis. The prevalence of malaria parasitemia among the population studied was 86.5%. There was no significant difference in the prevalence of parasitemia among the male and female sicklers (p=0.35). Malaria parasitemia was significantly associated with hematocrit <0.20 (p=0.03). There was no statistically significant difference between the type of prophylaxis used and parasitemia. In conclusion, the role of malaria prophylaxis in preventing parasitemia seems negligible. It is therefore recommended that more emphasis.

OBJECTIVES: To study the spectrum of hematological manifestations and evaluate the relationship between various hematological manifestations and CD4 cell counts in a hospital-based cohort of HIV-infected adults in and around Varanasi, North India.
MATERIALS-METHODS: The clinical and hematological profiles of the patients attending the Infectious Disease Clinic, Varanasi, India were recorded. The relationship between CD4 counts and various hematological manifestations was analyzed.
RESULTS: A total of 470 HIV-infected individuals were followed for 830 person years of observation (PYO). Rate of hematological episodes was 1047 episodes per 1000 PYO. CD4 counts were significantly lower in individuals with severe anemia and neutropenia compared to those without. However, no relation could be established between thrombocytopenia and CD4 counts. In the above- mentioned population, CD4 levels were significantly lower in those with anemia/neutropenia harboring any particular disease compared to those who had the same disease without anemia/ neutropenia.
CONCLUSIONS: There is a strong negative association between CD4 counts and the severity of anemia and neutropenia in this population. They can be considered as good clinical indicators to predict and access the underlying immune status. Though fall in the CD4 levels during neutropenia is observed, it is difficult to comment since the estimations of CD4 rely on the total leukocyte counts. However, the relation between anemia and disease progression is straight forward and quite useful for the treating physician.

The diagnosis of leukemia-specific mRNAs by polymerase chain reaction (PCR) and reverse transcription-PCR (RT-PCR) require well-known positive standard controls. In general, the positive controls are obtained from cell lines and leukemia patients who have been diagnosed at the molecular level by RT-PCR. These are expensive and restricted sources for standard positive controls. Thus, there is a need for less expensive and reproducible standard positive controls in this area. We have cloned the t (9: 22) p190, t (9: 22) p210, t (4: 11), t (1: 19), t (15: 17), t (12; 21) breakpoint junctions of fusion genes into the plasmids. Cloned fusion genes are suitable for testing PCR experiments of the molecular genetic diagnosis of leukemia samples. We cloned and optimized fusion gene junctions as a standard positive control to check PCR efficiency and as a standard positive marker for diagnosis.

Dysregulation of T-helper (Th)1 (IFN-g, IL-2, TNF-a) and Th2 (IL-4, IL-5, IL-10) type cytokines has been suggested in the pathogenesis of graft versus host disease (GvHD). We analyzed intracellular cytokine expression in Th (CD4+) lymphocytes in 23 patients undergoing allo-hematopoietic stem cell transplantation (HSCT) both in the week of neutrophil engraftment (S1) and on the posttransplant 100th day (S2). CD4+TNF-a+ cells increased from 22% at S1 to 26% at S2 in the allo-peripheral blood (PB) group. CD4+IL-2+ cells at S1 in the bone marrow (BM) group had a tendency to be higher than in the allo-PB group and also higher than at S2. IL-2 and TNF-a expressions at S1 were significantly higher and IL-2 expression at S2 was significantly lower in patients with grade II-IV acute GvHD compared with grade 0-I GvHD. IFN-g expression tended to be higher at S2 in the chronic GvHD group compared with the patient without GvHD. Consequently, the data in this study support the role of Th1 cytokines in GvHD.

Gemtuzumab ozogamicin (GO, MylotargTM) is an antibody-targeted chemotherapy agent that has been studied in acute myeloid leukemia (AML) at first relapse in adults. There is limited experience in pediatric patients. We report six patients with refractory/relapsed CD33+AML who were treated with GO on compassionate-use basis. One patient attained remission. One patient is still alive following hematopoietic stem cell transplantation (HSCT), and one patient died in remission. Two patients were refractory and three patients had a response with <5% blasts in the bone marrow. Fever and chills, hypotension and hypoxia were observed as side effects. Three patients developed veno-occlusive disease (VOD) of the liver. Two of these three patients had persistence of VOD at the time of their deaths. One patient treated postSCT had bone marrow response without VOD. GO should be used cautiously in chemotherapy-refractory AML pediatric patients due to the high incidence of VOD.

Hemophagocytic syndrome is characterized by fever, fatigue, weight loss, lymphadenopathy, and laboratory abnormalities including pancytopenia, liver dysfunction, hypertriglyceridemia and hyperfibrinemia. Histopathologically, lesions are characterized by mononuclear cell infiltration with marked histiocyte proliferation and phagocytosis of erythrocytes, leukocytes, platelets and their precursors by activated macrophages in the reticuloendothelial tissues. Hemophagocytic syndrome may develop from strong immunological stimuli such as severe infection, malignancy and autoimmune diseases.
We present a 73-year-old man with acute myeloblastic leukemia FAB M2 type (AML M2) whose bone marrow histology showed unusual hemophagocytosis by myeloid cells and myeloblasts.

Echinococcosis is a parasitic disease which occurs due to cyst hydatid Echinococcus granulosus and occurs in the liver in 55-70% of cases. Cyst hydatid treatment can be performed in three ways: primary surgery, medical and PAIR (Puncture Aspiration Injection Reaspiration) treatment. Medical treatment can be applied with PAIR in order to decrease post-surgery recurrence and facilitate the treatment in repeating cases. The most commonly used agent in medical treatment is albendazole. The most common albendazole-induced side effect is elevated levels of liver enzymes. It rarely causes bone marrow toxic consequences. We present herein a case with albendazole-induced leukopenia and thrombocytopenia, which to our knowledge has never been reported in the literature.

The first observation of homozygote Hb Q-Iran (α1 75(EF4) Asp-His) is presented in this report. The clinical and hematological data of the index case, his father and mother showed that homozygous or heterozygous Hb Q-Iran has no clinical importance.

Hb D-Ouled Rabah [beta19(B1)Asn>Lys] is a rare hemoglobin (Hb) beta chain variant reported from Tuareg tribes in Algeria and once from China. It was suggested that Hb D-Ouled Rabah might be specific of Berber-speaking populations. Our report describes the first observation of this hemoglobin variant in the Turkish population.