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   Table of Contents - Current issue
Coverpage
July-September 2019
Volume 10 | Issue 3
Page Nos. 77-109

Online since Friday, November 15, 2019

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REVIEW ARTICLE  

Management of adult immune thrombocytopenia: Recommendations by an expert Saudi panel Highly accessed article p. 77
Hazzaa Al-Zahrani, Aamer Aleem, Fahad Al Mohareb, Said Yousuf Ahmed, Ahmed M Al-Suliman, Hussain H Al Saeed, Mubarak S Al-Ghamdi, Hani Al-Hashmi
DOI:10.4103/joah.joah_51_19  
Immune thrombocytopenia (ITP) is a disorder characterized by an isolated thrombocytopenia in the absence of an identifiable cause. Management of ITP patients varies according to the clinical presentation, physicians' experience, availability of resources, and patient preferences. Currently, multiple therapeutic options are available for the management of chronic ITP and include splenectomy, rituximab, thrombopoietin-receptor agonists, and immunosuppressant agents. To develop a common approach on the management of adult ITP patients, a meeting of a panel of hematologists experienced in the management of ITP was convened. This review focuses on the expert opinion based on local experience in the field, in light of relevant literature and guidelines, and provides expert recommendations for the diagnosis and management of adult patients with ITP in Saudi Arabia.
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ORIGINAL ARTICLES Top

Prevalence of glucose-6-phosphate dehydrogenase deficiency and sickle-cell trait among blood donors: Revisited after 10 years in the same institute p. 84
Mansour S Aljabry, Abdulaziz Alhoshan, Fahad Alrawaf, Abdullah Alhowidi, Abdullah Alsahli, Saad Alrubaia, Abdulmajeed Alshaikh, Adnan Alkhayat, Mohammed Khalid Alabdulaali
DOI:10.4103/joah.joah_22_19  
BACKGROUND: Sickle cell anemia and glucose-6-phosphate dehydrogenase deficiency (G6PD) are the most common causes of inherited hemolytic anemia in Saudi Arabia. Due to high prevalence, some blood transfusion services perform routine blood donor screening for hemoglobin S (HbS) and G6PD enzyme assay. This study was conducted to reassess the prevalence of these disorders after 10 years of the last published report from the same institute. In addition, the utility of donor screening for sickle trait status was also reviewed. DESIGN: This cross-sectional study has utilized archived data of all blood donors between April 2016 and June 2016. All blood donors who fulfilled the inclusion criteria for blood donation during the study period were enrolled in this study. They all answered a standardized medical questionnaire and have been clinically examined by the blood bank medical staff as well. MATERIALS AND METHODS: All donors' samples were tested for sickle-cell trait (SCT) by HbS solubility test and capillary electrophoresis and for G6PD enzyme activity by fiuorescent spot test. All investigations were completed within 24 h after blood collection and processed based on the standardized procedures which have been fully accredited by the College of American Pathologists. RESULTS: Of the 2748 blood donors' samples, there were 2701 (98.3%) males and 47 (1.7%) females. SCT was present in 41 (1.49%), G6PD was detected in 80 (2.91%), and 14 (0.50%) donors were positive for both conditions. All the donors with positive results for both conditions were males. CONCLUSION: The utility of donor screening for SCT in the central region of KSA is questionable due to the low prevalence of SCT carrier among blood donors and increase of public awareness about HbS carrier status due to wide implementation of premarital screening program for sickle cell and thalassemia. In the same manner, screening of G6PD deficiency should be restricted only to high-risk recipient such as premature neonates.
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Effect of tertiary prophylaxis with low-dose factor VIII in quality of life in adult patients with severe hemophilia A p. 88
Prakas Kumar Mandal, Abhijit Phukan, Amrita Bhowmik, Debasis Gantait, Prantar Chakrabarti
DOI:10.4103/joah.joah_37_19  
BACKGROUND: Hemophilia A, characterized by deficiency of factor VIII (FVIII), is an X-linked bleeding disorder. It is graded as mild, moderate, or severe based on the FVIII level in plasma. Bleeding-related complications are often associated with severe form of the disease. Prophylaxis has now become the standard of care for severe hemophilia A patients. OBJECTIVES: The study aimed to describe the effect of tertiary prophylaxis with low dose FVIII in quality of life in adult patients with severe hemophilia A. SUBJECTS AND METHODS: A random 20 adult severe hemophilia A male patients aged 18–40 years with joint deformities, were included for this study. Long-acting recombinant FVIII, Fc Fusion protein (ELOCTATE, Biogen) was then given twice weekly at a dose of 15 IU/Kg/dose twice weekly for 3 months. Results were compared with the previous 3 months records of no prophylaxis. Efficacy was determined in terms of annualized bleed rate (ABR ) and absenteeism from work during this study period. Inhibitor screening was done at regular intervals during prophylaxis. RESULTS: There was significant decrease in mean ABR along with absenteeism from work during prophylaxis as compared to on-demand therapy (3.6 bleed/year and 9.4 days/year compared to 37.8 bleed/year and 64.7 days/year, respectively). The mean FVIII requirement during prophylaxis (1560 IU/kg) was also less than the on-demand therapy (2150 IU/Kg/year). Majority (85%) had a FVIII trough level of <1%. Only one patient complained of myalgia while on prophylaxis. No inhibitor development was found in any of the individuals. CONCLUSION: Low-dose recombinant long-acting FVIII prophylaxis can achieve a significantly lower bleeding rate in adult hemophilia A patients along with improved quality of life.
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Pentazocine addiction among sickle cell disease patients and perception of its use among health-care workers p. 94
Akinsegun Akinbami, Ola Bola, Ebele Uche, Mulikat Badiru, Olusola Olowoselu, Aishatu Maude Suleiman, Benjamin Augustine
DOI:10.4103/joah.joah_39_19  
BACKGROUND: Prolonged use of pentazocine in sickle cell disease (SCD) because of chronic pain may result in mental dependence (addiction) and/or physical dependence leading to withdrawal symptoms on suddenly stopping its use. This study was aimed at determining the prevalence of pentazocine addiction among SCD patients and health-care worker (HCW) perception on its use. MATERIALS AND METHODS: This was an interviewer-administered, questionnaire-based, cross-sectional study. The study involved clients attending the Lagos State University Teaching Hospital sickle cell clinic and the hospital HCWs. Consenting participants filled a World Health Organization structured questionnaire developed and extracted from ASSIST which is A - Alcohol, S - Smoking and S - Substance, I - Involvement, S - Screening, and T - Test. The HCWs were evaluated using a pretested, validated questionnaire. RESULTS: A total of 350 participants were recruited consisting of 169 (48.3%) males and 181 (51.7%) females. ASSIST report showed 88% of them had low score of 0–3, 10% had moderate score of 4–26, while 2% (7 of 350) had high score of >27. A total of 61 HCWs were interviewed, and 18% and 8.2% of them believed 40%–60% and more than 60%, respectively, of the SCD patients were addictive to pentazocine. CONCLUSION: While the issue of drug addiction should not be ignored, the appropriate treatment of SCD patients in Nigeria who are prevented from getting high-quality care should be appropriately addressed. The risk of addiction is overestimated among HCW.
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CASE REPORTS Top

De novo acute myeloid leukemia with t(8;21)(q22;q22) and monosomy 7 p. 99
Harish Kumar, Paresh Singhal, Ajay Malik, Sanjeevan Sharma
DOI:10.4103/joah.joah_40_19  
The t(8: 21)(q22;q22) is the most common cytogenetic abnormality, usually with a favorable risk, in acute myeloid leukemia (AML). This translocation is not only of diagnostic significance but also has impact on survival outcomes and therapeutic implications. However, patients with adverse outcome in this category of recurrent genetic abnormalities have additional cytogenetic/molecular aberrations with elevated white blood cells count, CD56 expression, and extramedullary manifestations. This case is reported with aim to describe an elderly female who had a sudden downhill clinical course in de novo AML-FAB class M2 in spite of t(8;21), which was associated with monosomy 7 as an additional chromosomal abnormality and absence of high risk clinically relevant genetic mutations.
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RAB27A mutation in a patient with griscelli syndrome type 2, successfully cured by hematopoietic stem cell transplantation: Sustained remission p. 103
Abdullah A Baothman, Fatima Mehdawi, Loie Goronfolah, Hani Almalki
DOI:10.4103/joah.joah_34_19  
Griscelli syndrome (GS) is a rare autosomal recessive disorder. It features with clinical constellation of skin hypopigmentation, silvery-gray hair, associated with neurological involvement (type I), or defective cell-mediated immunity with hemophagocytosis (type II) that leads to recurrent infection, and patients succumb to death in early childhood or isolated “quiet” GS (type III). To our knowledge, more than sixty patients have been reported mainly from the Mediterranean region with the first reported Saudi cases, reported by Harfi et al. in 1992. The outcome of GS is guarded if not treated early due to recurrent fulminant infections, disease recurrence, and the impending severity of central nervous system disease. Hematopoietic stem cell transplantation (HSCT) is the single current therapeutic treatment for GS II. HSCT, therefore, should be performed as early as possible to prevent potential complications. The reported patient had sustained remission and normal hematopoietic function after allogeneic bone marrow transplant from a human leukocyte antigen-identical sibling donor. GS II syndrome should be considered in any child with hypopigmented skin, silvery-gray hair, and primary immunodeficiency. Early recognition and early HSCT intervention are associated with favorable outcome.
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IMAGE IN HEMATOLOGY Top

A case of iga-kappa multiple myeloma with intracytoplasmic Auer rod-like inclusions p. 106
Sawsen Bouzidi, Aman Allah Nasr, Selim Bouzguenda, Samy Layouni, Najiba Fekih-Mrissa, Fehmi Msadek, Brahim Nsiri
DOI:10.4103/joah.joah_43_19  
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Plasma cell leukemia mimicking hairy cell leukemia: Extended role of immunophenotyping in correct diagnosis p. 108
Ayesha Majeed Memon, Natasha Ali
DOI:10.4103/joah.joah_26_19  
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