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   2014| October-December  | Volume 5 | Issue 4  
    Online since December 13, 2014

 
 
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REVIEW ARTICLE
Management of myelodysplastic syndromes: Expert consensus opinion from the Saudi MDS Working Group
Ahmed Alaskar, Abdul Kareem Al Momen, Ahmad Al-Saeed, Ahmed Al Sagheir, Amr Hanbali, Ayman Al-Hejazi, Hani Al-Hashmi, Khalid Al-Anazi, Mohsen Al Zahrani, Saud Abu Harbesh, Zayed Al-Zahrani
October-December 2014, 5(4):123-132
DOI:10.4103/1658-5127.146946  
Myelodysplastic syndromes (MDSs) constitute a heterogeneous group of clonal hematopoietic disorders. A panel of Saudi hematologists representing the Saudi MDS Working Group convened with two international experts to develop the guidelines for MDS diagnosis and treatment. The recommendations were formulated on the basis of a list of real cases and therapy-related questions. The diagnostic procedures should help distinguish MDS from other causes of cytopenia and dysplasia and other clonal stem cell disorders. Blood smear, bone marrow aspirate and biopsy, and cytogenetic testing are among the mandatory diagnostic tests in MDS. Higher resolution genetic testing like mutational analysis and single nucleotide polymorphisms can be suggested for the workup depending on the clinical condition and availability of these technologies. The Working Group stressed that the heterogeneity of MDS strongly withstands a risk-adapted treatment strategy based on the international prognostic scoring system risk group of patients.
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CASE REPORTS
Tuberculosis and autoimmune hemolytic anemia: Case report and literature review
Hussain Bahbahani, Maryam Al-Rashed, Mohammed Almahmeed
October-December 2014, 5(4):164-167
DOI:10.4103/1658-5127.146953  
Tuberculosis (TB) is a mycobacterial infectious disease that can affect any organ system. The wide varieties of manifestations that TB can present with make the disease a challenge for treating physicians. Anemia is commonly seen in patients with TB, and it is usually anemia of chronic disease. However, the occurrence of autoimmune hemolytic anemia in the setting of TB infection is rare.
  5,909 598 3
ORIGINAL ARTICLES
Clinical and laboratory presentations of Janus Kinase II-positive patients in Saudi population
H Malhan, H Alzahrani, Ghada M Elgohary, T Owaidah, M Aslam, Said Yousuf Mohamed
October-December 2014, 5(4):133-140
DOI:10.4103/1658-5127.146947  
Introduction: Janus Kinase II (JAK-II) mutation has a pivotal role in the pathogenesis of the myeloproliferative neoplasms (MPN) and has been shown to be involved in thrombotic complications of these diseases. However, there are limited data regarding clinic-biological features and cytogenetic abnormalities in the Saudi population. Aim: The main aim of this study was to examine clinical presentations, laboratory findings and prognosis of JAK-II-positive Saudi population referred to KFSHRC for testing for variable indications. Methods: A total of 200 patients was referred to our institute for JAK-II mutation testing by polymerase chain reaction based on clinical/laboratory suspicion; about 102 (51%) proved positive. Of the positive cohort, only 62 patients had completed evaluable medical records. Results: Their mean age was 48.2 (range: 16-81) years and 51.6% were males. About 75% had symptoms such as headache 13 [21%]; pruritus 9 [14.5%], visual 7 [11.3%], erythromelalgia 2 [3.2%], and none specific symptoms like fatigue/weakness abdominal discomfort in 30 (48.2%) while the remaining 25% were diagnosed incidentally from abnormal complete blood cells. Clinic-radiological features included: Splenomegaly (53.6%), hepatomegaly (24%), and skin lesions (8%). Thrombosis of portal, hepatic, splenic and mesenteric veins was seen in 24%, stroke and transient ischemic attack in 14%, deep vein thrombosis and pulmonary embolism in 11.2%, ischemic heart disease in 9.6% and 3.2% had arterial thrombosis. The mean peripheral blood count showed white blood cell 13.3 × 109/l, hemoglobin 147.3 g/l and platelets 714 × 109/l. Of the 35 patients evaluated by bone marrow biopsy, the majority (80%) had hyperculluarity. Cytogenetic abnormalities were found in 7 (20%) of the 35 patients and one patient had both JAK-II and BCR/ABL mutations. All the positive patients except 3 (4.8%) were diagnosed as MPNs, 29 (46.7%) polycythemia vera, 24 (38.7%) essential thrombocythemia, 2 (3.2%) primary myelofibrosis and MPN-not otherwise specified in 1 (1.6%). Treatment modalities including hydroxyurea, anagrelide, and thalidomide were used in 75%, antiplatelets were used in nearly 2/3 (61.3%) and 29% of patients required anticoagulants. Three-year actuarial survival was 87% with death related mainly to progressive fibrosis and cytopenia, thrombotic complications necessitating major surgeries, pneumonia or sepsis.
  2,498 1,589 -
Prognostic value of 13q14 deletion and IgH 14q32 rearrangement by interphase fluorescence in situ hybridization in patients with multiple myeloma
Nevine A Kassem, Dahlia A Elswefy, Nermine I Eman, Mahira I Mogy, Mohamed M Moussa, Rania N Ali
October-December 2014, 5(4):141-147
DOI:10.4103/1658-5127.146948  
Background: Multiple myeloma (MM) is a clonal bone marrow (BM) disease characterized by the neoplastic transformation of differentiated B cells with the accumulation of malignant plasma cells in the BM compartment. In Egypt, its extrapolated prevalence is 17,630/76,117,421 with annual rate around 4,085/76,117,421. Chromosomal aberrations in MM are typically complex and represent a hallmark of the disease. The most frequent structural aberrations are 13q14 deletion detected in 33-50% of the cases and 14q32 IgH rearrangement detected in 60-70% of the cases with t (11;14) (q13;q32) being the most frequently detected 14q32 translocation seen in 15% of these patients. Aim: The aim was to detect both 13q14 deletion and 14q32 IgH rearrangement by interphase fluorescence in situ hybridization (I-FISH) on 100 newly diagnosed Egyptian myeloma patients and their relation to the patients' outcome and prognosis. Subjects and Methods: Patients were subjected to a complete history, clinical examination, and laboratory. We have also tested the expression of 13q14 deletion by locus-specific identifier (LSI) D13S319 probe assay and 14q32 IgH rearrangement by IgH dual color FISH break-apart assay on BM aspirates collected from the patients at diagnosis (before starting therapy). Staging and follow-up of the patients were carried out to detect the outcome of the disease. Results: We found that 65% of the examined myeloma patients showed genetic aberrations (13q14 deletion, 14q32 IgH rearrangement or both) by I-FISH. Of which, 40% were positive only for 13q14 deletion, 20% were positive only for 14q32 IgH rearrangement and only 5% were positive for both aberrations. 13q14 deletion was associated with more patients being resistant to chemotherapy or dying indicating its poor impact on patients' prognosis, whereas 14q32 IgH rearrangement was associated with more patients in remission indicating its good impact on patients' prognosis. Comparing myeloma patients who went into remission to those who died or were resistant to chemotherapy after receiving treatment, revealed highly statistical significance in these patients (P ≤ 0.001) as regards to their serum calcium, albumin, B2 microglobulin, and BM clonal plasma cell levels with P values (0.0009, 0.0001, 0.0001 and 0.0001, respectively). Moreover, a statistical significance (P ≤ 0.05) was found in these patients as regards to their total protein and serum M-component with P values (0.0288 and 0.0182, respectively). Summary/Conclusion: 13q14 deletion detected by LSI D13S319 FISH probe in myeloma patients was seen in 40% of the studied cases and was associated with patients being resistant to chemotherapy or dying. Whereas, 14q32 IgH rearrangement detected by IgH dual color break-apart FISH assay in these patients was seen in 20% of the studied cases and was associated with patients in remission.
  3,495 586 -
CASE REPORTS
Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis
Sadia Sultan, Syed Mohammad Irfan
October-December 2014, 5(4):161-163
DOI:10.4103/1658-5127.146952  
Pure white cell aplasia (PWCA) is an exceedingly rare hematological disease of unknown origin exemplified by agranulocytosis, virtually lack of all granulocytic spectrums (from myeloblast to neutrophils) in the bone marrow with normal erythroid and megakaryocytic lineages. It has been associated with underlying autoimmune disorders, thymomas, chronic lymphocytic leukemia and drug induced or may be idiopathic. Here we report the case of a patient with this rare finding, who was admitted to our hospital with septicemia having agranulocytosis, multiple oral candidiasis, perianal abscess and right arm cellulitis. Bone marrow examination revealed absolute absence of all myeloid precursors cells. All diagnostic workup for secondary underlying cause were found to be negative and diagnosed as idiopathic PWCA. Immunosuppression with methylprednisolone pulse therapy was failed to induce neutrophils recovery. PWCA is a benign disorder, but have serious consequences due to threatening febrile neutropenia, disseminated fungal infection and septicemia. Needs urgent management depending upon underlying etiology.
  2,991 235 -
ORIGINAL ARTICLES
Serum ferritin level in adult sickle cell anemia in Saudi population
Ahmed M Alsuliman, Mahmoud Albagshi, Kefah B Algadeeb, Abdulmottaleb Aldanden, Abbas I Alabdultif
October-December 2014, 5(4):148-150
DOI:10.4103/1658-5127.146949  
Background: Vaso-occlusive and hemolysis are the clinical hallmarks of sickle cell disease .patients with sickle cell at risk of iron overload due to chronic blood transfusion treating complications of the disease ,in addition to increasing iron absorption from gut and chronic hemolysis. Material and Methods: This is retrospective study on total of 174 adult sickle cell disease patients were included in this study, attending hematology clinic in king Fahad Hofuf hospital and inherited blood disorder center-Alhassa. for each patient demographic data was obtained (age, sex, nationality), history of previous blood transfusion, hemoglobin level, serum ferritin level. In total, 174 naïve Saudi adult patients (113 males, 61 females) were included in this study. Mean age was 28.2 ±10 years (range 12- 62 years), sex ratio (M/F) was 1.8 :1.0 . 93 (53%) patients had history of previous blood transfusion. The overall mean serum ferritin concentration was 587 ± 547(18.49 - 2660 ng\dl). The mean serum ferritin level for male is 649 ± 606 ng\dl and mean serum ferritin level for female is 473± 440ng\dl. Conclusion: Iron overload in sickle cell disease patients from eastern province remains mild to moderate, with significant sex difference. The repetitive assessment of serum ferritin level should be consider to sickle cell patients whom frequent blood transfusion is needed like stroke and renal failure.
  2,109 267 -
CASE REPORTS
Successful outcome of postsplenectomy chemotherapy in an acute myeloid leukemia patient with massive bilharzial splenomegaly
Shahid Iqbal, Syed Zaidi, Khalid Al-Mohaimeed, Imran Tailor, Ibraheem Almotabi, Nawal Al-Shehry, Assem El-Ghazaly, Samer Al-mudaibegh, Mamoun Ibrahim, Karrar El-Hussein, Abdulaziz Al-Humaidi
October-December 2014, 5(4):156-160
DOI:10.4103/1658-5127.146951  
Bilharzial hepatic fibrosis is usually accompanied by marked enlargement of the spleen. Schistosoma hematobium and Schistosoma mansoni infestation is endemic in Africa and southern part of Saudi Arabia. Patients with acute myeloid leukemia (AML) present with clinical features resulting from bone marrow failure, symptoms resulting from organ infiltration with leukemic cells (including splenomegaly), or both. Co-occurrence of bilharzial splenomegaly and AML is intuitively possible. Spontaneous splenic rupture has been reported to be catastrophic in both conditions and rarely splenic rupture may be the first manifestation of AML. Splenectomy is a considerable option in symptomatic splenomegaly in schistosomiasis. An enlarged spleen may also serve as a sanctuary site for residual leukemic cells after chemotherapy. Splenectomy has been reported in pediatric AML after chemotherapy and selected adult patients with myelodysplastic syndrome and myeloproliferative neoplasm (MPN). However, to the best of our knowledge, this is the first report of successful outcome of splenectomy prior to induction chemotherapy in an AML patient who presented with huge symptomatic splenomegaly due to past history of schistosomiasis, had also shown refractoriness to platelet transfusion due to hypersplenism and vaginal bleeding. Splenectomy in such a scenario was a difficult decision but was feasible with adequate expertise, vigilance and blood products availability. The patient is alive in complete remission with good performance status more than 2½ years after therapy.
  2,046 150 -
ORIGINAL ARTICLES
Study of CD123 (interleukin-3 receptor alpha Chain) and nuclear factor kappa B in adult acute myeloid leukemia patients
Inas Asfour, Soha Yoyssef, Maryse Ayoub, Nevine Moustafa, Hani Ayash, Tussneem Elhassan, Ghada M ElGohary
October-December 2014, 5(4):151-155
DOI:10.4103/1658-5127.146950  
Background: The genomic alterations in acute myeloid leukemia (AML) affect the function of signaling molecules, transcription factors, and growth factor receptors besides they influence the response to treatment. Interleukin-3 receptor alpha chain (CD123) is overexpressed in about 45% of AML, this phenomenon was associated with high blast cell counts at diagnosis, with a worse prognosis. The transcription nuclear factor kappa B (NF-κB) can intervene in oncogenesis through its capacity to regulate the expression of a large number of genes that regulate apoptosis, cell proliferation, and differentiation. This study aimed to assess the relationship between CD123 expression and NF-κB level, in adult patients with AML at presentation and after induction therapy together with a well-known diagnostic and prognostic factors in AML. The study was conducted on forty adult newly diagnosed AML (group A) as well as twenty adult subjects who were hemato-oncologically free, as a control group (group B). Bone marrow (BM) examination and immunophenotyping by flow cytometry (FCM) on BM aspirate, for CD123 and quantitation of NF-κB in BM samples using ELISA (at three levels: Plasma, cytoplasmic and nuclear fraction of blasts, accordingly cytoplasmic/nuclear ratio) were performed for all studied subjects. In addition, levels of CD123 on blast cells and NF-κB were evaluated at D28 for remitted and resistant cases. All patients were positive for CD123 by FCM on BM blasts at time of diagnosis. The median cell counts expressing CD123 was 52.9 (34.4-75.6). Positive expression of CD123 was ≥20%, while in the control subjects CD123 expression was 25.1 (16.9-59.3), with significant decrease in CD123 expression after therapy, Besides, CD123 was also significantly higher in resistant patients compared to remitted patients (P = 0.009). As regard NF-κB, it was significantly higher in AML patients compared to control subjects at the three levels (plasma - cytoplasmic fraction - nuclear fraction) with a significant decrease after therapy. It was found that those NF-κB levels detected at follow-up (D28) on BM expression were significantly higher in resistant patients compared to remitted patients. While only The follow-up results, showed a highly significant positive correlation between CD123 expression on blast cells level and NF-κB plasma level alone (r = 0.587, P = 0.007) while there was not any correlation between CD123 and other two NF-κB levels neither at diagnosis nor at the follow-up. Conclusion: These data suggest that the correlation between CD123 and NF-κB was identified in variable results, and further elucidation of this role is likely to have important implications.
  1,830 232 -
IMAGE IN HEMATOLOGY
Wandering (ectopic) spleen
Hazza Alzahrani, N Chaudary, Rana AlQahtani
October-December 2014, 5(4):168-168
DOI:10.4103/1658-5127.146954  
  1,132 106 -
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