E-ISSN: 1308-5263
Turkish Journal of Hematology - Turk J Hematol: 25 (3)
Volume: 25  Issue: 3 - 2008
REVIEW
1. Hepatic complications of allogeneic hematopoietic cell transplantation
Mutlu Arat, Ramazan Idilman
Pages 111 - 123
Hepatic complications of allogeneic hematopoietic cell transplantation contribute substantially to the overall success of the procedure and represent a major cause of morbidity and mortality. Early hepatic complications consist of the sinusoidal obstruction syndrome, drug toxicities, infections, acute graft-versus-host disease, while late hepatic complications consist of chronic graft-versus host disease, chronic viral hepatitis, and iron overload states. Successful management of the hepatic complications of allogeneic hematopoietic cell transplantation is dependent on several factors. These include the recognition and elimination of any pre-transplant risk factors for these problems and the development of strategies to evaluate and prevent them in both the early and later posttransplant periods.
The aims of present review are 1) to identify the early and late hepatic complications of allogeneic hematopoietic cell transplantation, in the chronological order at which they occur, 2) to characterize the diagnostic procedures used to identify them, and finally 3) to present the current therapeutic approaches used to manage these problems.

RESEARCH ARTICLE
2. Minimal Residual Disease (MRD) Detection with Translocations and T-Cell Receptor and Immunoglobulin Gene Rearrangements in Adult Acute Lymphoblastic Leukaemia Patients: A Pilot Study
Müge Sayitoğlu, M.cem Ar, Özden Hatırnaz, Şeniz Öngören, Ümit Üre, Zafer Başlar, Sema Sırma, Yıldız Aydın, Uğur Özbek, Burhan Ferhanoğlu
Pages 124 - 132
Monitoring minimal residual disease has become increasingly important in clinical practice of ALL management. Break-point fusion regions of leukaemia related chromosomal aberrations and rearranged immunoglobulin (Ig) and T cell-receptor (TCR) genes, which can be detected by polymerase chain reaction (PCR), are used as leukaemia specific markers in genetic studies of MRD. A total of 31 consecutive patients with newly diagnosed ALL were screened for eligibility criteria. Of those 26 were included in the study. One patient with partial response following induction therapy and four patients who were lost to follow-up after induction were excluded from the study; thus 21 patients were evaluated for MRD. Chromosomal aberrations were detected in 5 (24%) of the patients and were used for MRD monitoring. Three patients had t(9;22) translocation, the other 2 had t(4;11) and t(1;19). MRD-based risk stratification of the 16 patients analysed for Ig/TCR rearrangements revealed 3 low-risk, 11 intermediate-risk and 2 high-risk patients. MRD monitoring is progressively getting to be a more important predictive factor in adult ALL patients. As reported by others confirmed by our limited data there is a good correlation between MRD status and clinical outcome in patients receiving chemotherapy. The pilot-study presented here is the first that systematically and consecutively performs a molecular MRD monitoring of ALL patients in Turkey.

3. Protein Z G79A polymorphism in Turkish pediatriccerebral infarct patients
Ayşenur Öztürk, Yonca Eğin, Gülhis Deda, Serap Teber, Nejat Akar
Pages 133 - 135
Protein Z (PZ) plays an enhancer role in coagulation as an anticoagulant. This is the first study in which G79A polymorphism investigated in Turkish paediatric stroke patients. Ninety-one paediatric stroke patients with cerebral ischemia and 70 control subjects were analyzed for PZ G79A and also FVL, PT mutations. PZ 79 ‘A’ allele in homozygous state was found in five patients (5,5%), while it was found only in one control subject (1,4%) and it was seemed as a risk factor for peadiatric ischemia [OR=3,94 (0,44-35,1)]. When patients and controls who had FVL and PT carriers were excluded, AA genotype carried a risk [OR=3,88 (0,41-36,5)]. Also plasma protein Z levels measured in 21 stroke patients and 52 controls. Plasma protein Z levels were not different between stroke patients (500,95 ngmL-1±158,35) and controls (447,34 ngmL-1±165,97). But the plasma levels of protein Z was decreased in patients with AA genotype. Our data showed that carrying 79 AA genotype could be a genetic risk factor for cerebral infarct in peadiatric patients.

4. Insulin-like growth factor-1 levels in children with Beta-thalassemia minor
Hamdollah Karamifar, Mehran Karimi, Nargrs Sobhani
Pages 136 - 139
OBJECTIVE: Growth retardation in children with b-thalassemia major is multifactorial. Some etiologies described for this condition are hemochromatosis, disturbed growth hormone (GH) / insulin growth factor-1 (IGF-1) axis, undernutrition and hypermetabolism. It has also been proven that growth retardation is present in b-thalassemia major children despite
regular transfusion and chelation. Our aim was to evaluate the level of IGF-1 in b-thalassemia minor subjects and compare it with that in healthy children.
METHODS: Fifty children aged 6 months to 15 years with b-thalassemia minor (32 males, 18 females) and 50 age- and sex-matched normal healthy children were selected. Medical history was taken and complete physical examination was done in each case; IGF-1 level was checked in all cases. This study was done in Shiraz, southern Iran, during 2005.
RESULTS: IGF-1 levels were significantly lower in b-thalassemia minor children than normal children (P = 0.015). This result demonstrates that some etiologies of growth failure in b-thalassemia major other than those described to date can exist, which may be shared with b-thalassemia minor in feature or may be transformed by genes that are either expressed or not.
CONCLUSION: We conclude that in addition to that observed in b-thalassemia major, IGF-1 level is also decreased in
b-thalassemia minor, and these two may have similar etiologies

5. Hematological disorders in 6-hydroxydopamine-induced rat model of Parkinson’s disease
Lucian Hritcu
Pages 140 - 144
OBJECTIVE: The present work was undertaken in order to investigate the effects of right-unilateral lesion of substantia nigra neurons by means of 6- hydroxydopamine (6-OHDA), a dopaminergic-selective neurotoxin, on hematological parameters in rats. The primary reason for the using of rat model of Parkinson’s disease was the interest regarding the role of the central dopaminergic system in hematopoiesis regulation because some neurological diseases like Parkinson’s disease are well-correlated with anemia associated with autonomic dysfunction in rats.
METHODS: Thirty male Wistar rats weighing 200 ± 50 g at the start of the experiment were used. The substantia nigra was right-unilateral lesioned by stereotaxic microinjections of 8 micrograms (free base) 6-OHDA, dissolved in 4 µl physiological saline containing 0.1% ascorbic acid, administered through the Hamilton microsyringe over 4.50 minutes. 7 days after neurosurgery, we assessed the total number of white blood cells (WBC), the total number of red blood cells (RBC), hemoglobin level and the erythrocyte indexes (mean cell volume, MCV and mean cell hemoglobin, MCH).
Hematological parameters were assayed by a COULTER® Ac◊T 5diff CP-precision instruments for hematology research.

RESULTS: 6-OHDA treatment induced a significantly decrease of white blood cells (p<0.03), red blood cells (p<0.01), hemoglobin level (p<0.02) comparative with sham-operated rats. By contrast, in the 6-OHDA-lesioned rats the erythrocyte indexes (mean cell volume, MCV (p<0.04); mean cell hemoglobin, MCH (p<0.01)) were significantly enhanced comparative with sham-operated rats.
CONCLUSION: On the whole, the obtained data indicate the important role of the central dopaminergic system in the regulation of erythrocyte dynamics.

6. Immunohematological characteristics of Nigerian sickle cell disease patients with osteomyelitis
Elkanah Ayodele Orimolade, Lateef Salawu
Pages 145 - 148
OBJECTIVE: We aimed in this paper to investigate some immunohematological characteristics of Nigerian sickle cell anemia (SCA) patients with osteomyelitis.
METHODS: Thirty SCA patients with osteomyelitis (SO) and 30 SCA patients without osteomyelitis (S) were investigated. The PCV, WBC and platelet count were done on automated counter, while the erytorocyte sedimentation rate (ESR) was determined by Westergren’s technique. C3 activator, C1-INH, IgA, IgG and IgM were estimated by the single radial immunodiffusion method.
RESULTS: The SO patients weighed less (z =1.943, p<0.055) and were shorter (z = -2.064, p<0.039). High serum levels of IgG, IgM, C1-INH and C3 activator were also found in the SO group. ESR correlated positively with hematocrit (r=0.371, p<0.04) and C3 activator (r=0.468, p<0.03) in SO. Similarly, WBC correlated positively with C1-INH
(r=0.806, p<0.01), while we also noted positive correlation between C1-INH and C3 activator (r=0.525, p<0.02). In SO, ESR correlated positively with both IgM (r=0.531, p<0.02) and C3 activator (r=0.449, p<0.05).

CONCLUSION: This study suggests some derangement in immune status in Nigerian SCA patients with osteomyelitis and that C1-INH and C3 activator may be useful markers of immune status in SCA patients.

CASE REPORT
7. De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
Rakhee Kar, Rajat Kumar, Seema Tyagi
Pages 149 - 151
B-cell prolymphocytic leukemia (B-PLL) comprises 1% of chronic lymphocytic leukemias. CD5 positivity is seen in 1/3rd of cases which generally arise from pre existing CLL. They have longer median survival compared with de novo B-PLL which are commonly CD5 negative and are more aggressive with an older age of presentation. Herewith, we describe a 48-year-old male of de-novo CD5+ B-PLL presenting with minimal lymphadenopathy and massive splenomegaly with 90% atypical lymphoid cells in the peripheral smear and bone marrow. Immunophenotyping was strongly positive for CD5, CD45, CD19, CD22, FMC-7, S-Ig and CD38, moderately positive for CD 11c, weakly positive for CD23 and negative for CD-103 and ZAP 70. The patient responded well to fludarabine and cyclophosphamide and had an uneventful hospital course. Our case illustrates a de-novo B-PLL with aberrant CD5 positivity who had a short duration of illness, younger age at presentation and favourable treatment outcome.

8. A childhood acute lymphoblastic leukemia (ALL) case with t(3;17)(q23;p13),t(5;12)(q31;p13),inv(11)(p15q12)
Dilhan Kuru, Flükriye Yılmaz, Su Gülsün Berra, Cengiz Canpolat, Seniha Hacıhanefioğlu, Yelda Tarkan Argüden, Ayhan Deviren, Ayşe Çırakoğlu
Pages 152 - 154
It is known that clonal chromosomal changes in childhood ALL are nonrandom and important markers for diagnosis, prognosis and relaps. In this report we present 4 year-old boy with ALL-L1 who has complex chromosomal rearrangements. Chromosome analysis was performed on bone marrow aspiration sample in relaps after one year from diagnosis and induction chemotherapy. The karyotype was; 46,XY,t(3;17)(q23;p13),t(5;12)(q31;p13),inv(11)(p15q12) [11]/46,XY[8]

LETTER TO EDITOR
9. A case of adult acute T-cell lymphoblastic leukemia presented with hemophagocytic syndrome
Ahmet Ifran, Fahri Şahin, Kürşat Kaptan, Mükerrem Safalı, Cengiz Beyan
Pages 155 - 156
Abstract |Full Text PDF

10. Chronic Myeloid Leukemia Associated With Signet-Ringed Adenocarcinoma of Stomach And Review Of The Literature
Gönenç Kocabay, Betül Tiryaki, Kamil Yazıcı, Mustafa Yenerel
Pages 157 - 158
Abstract |Full Text PDF

11. Author Reply
Abdul Rehman
Pages 159 - 160
Abstract |Full Text PDF

IMAGES IN HEMATOLOGY
12. A case of multiple myeloma with double malarial infection
Harish Chandra, Smita Chandra
Pages 161 - 162
Abstract |Full Text PDF

NEWS
13. Obituary
Nejat Akar
Page 163
Abstract |Full Text PDF