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   Table of Contents - Current issue
Coverpage
April-June 2019
Volume 10 | Issue 2
Page Nos. 37-75

Online since Thursday, July 11, 2019

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REVIEW ARTICLE  

Multiple myeloma in Saudi Arabia: Consensus of the Saudi lymphoma/myeloma group Highly accessed article p. 37
Ahmed Alaskar, Ahmad Alsaeed, Fahad Z Alsharif, Hani Alhashmi, Mubarak Alghamdi
DOI:10.4103/joah.joah_62_18  
Multiple myeloma (MM) is a relatively uncommon cancer in Saudi Arabia. It is estimated that MM and lymphomas accounted for 9.6%–11% of cancer-related deaths in the Kingdom in 2014. However, published data on the characteristics and treatment patterns of, and outcomes for, MM patients in Saudi Arabia are scant. The Saudi Lymphoma/Myeloma held the “Scientific Gap Analysis Advisory Board” meeting in Riyadh on January 26, 2018, which represented six Saudi specialized institutions, in order to discuss different aspects of MM management in Saudi Arabia and to reach a consensus statement in each aspect of MM in Saudi Arabia. This consensus meeting brought together a panel of Saudi experts in MM to share their views on current trends and practice in Saudi Arabia and how these compare with the global picture.
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ORIGINAL ARTICLES Top

Pattern of neutrophil-lymphocyte ratio and platelet-lymphocyte ratio in sickle cell anemia patients at steady state and vaso-occlusive crisis p. 45
Adekunle Emmanuel Alagbe, John Ayodele Olaniyi
DOI:10.4103/joah.joah_57_18  
BACKGROUND: Neutrophil-lymphocyte ratio (NLR) and Platelet-Lymphocyte ratio (PLR) are emerging markers of systemic inflammation in chronic disorders, including hemoglobinopathies. OBJECTIVE: This study determined the pattern of NLR and PLR in sickle cell anemia (SCA) patients during steady state and vasoocclusive crisis (VOC). MATERIALS AND METHODS: A cross-sectional study composed of 60 adult SCA patients (30 during VOC and 30 during steady state). Thirty apparently healthy hemoglobin A (HbA) age- and sex-matched controls were included in the study. Complete blood count (CBC) was done for each participant; NLR was calculated as absolute neutrophil count (ANC) divided by the absolute lymphocyte count (ALC), and PLR was calculated as platelet count divided by ALC. STATISTICAL ANALYSIS: Data were analyzed using SPSS (version 22), and the results were considered statistically significant if value of P < 0.05. RESULTS: Total white-cell count, ANC, ALC, platelet count, and NLR were significantly higher in SCA patients than those of HbA controls. Total white-cell count, ANC, and NLR were significantly higher in the VOC than the steady-state group. PLR was not statistically different between the steady state and the VOC groups. In both groups of SCA patients, NLR was positively correlated with PLR, while percentage fetal hemoglobin was negatively correlated with percentage hemoglobin S. Hematocrit was negatively correlated with ANC and ALC in SCA patients. CONCLUSION: This study showed a significantly higher NLR and PLR in SCA than the control group. These are reflective of the roles of neutrophils, platelets, and lymphocytes in the constitutive inflammatory process. These ratios could be adopted for use in SCA patients because these markers are cheap, stable, and easily derivable from CBC.
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Fluorescence In situ hybridization signal patterns and intrachromosomal breakpoint cluster region-abelson murine leukemia viral oncogene homolog 1 amplification analysis in imatinib-resistant chronic myelogenous leukemia patients using tricolor dual fusion probe p. 51
Karthik B K. Bommannan, Shano Naseem, Neelam Varma, Jogeshwar Binota, Pankaj Malhotra, Subhash Varma
DOI:10.4103/joah.joah_69_18  
BACKGROUND: Cytogenetic evaluation is required till a complete cytogenetic remission is achieved in chronic myelogenous leukemia (CML) patients on tyrosine kinase inhibitor (TKI) therapy. The routine dual colour fluorescence in situ hybridization (FISH) probes are less sensitive in identifying der(9) abnormalities. BCR/ABL/ASS1 tri-colour dual fusion (TCDF) probe is highly sensitive and specific in identifying der(9) deletions and random signal overlaps. METHODS: Peripheral blood interphase FISH analysis was performed on imatinib-resistant CML patients using TCDF probe. RESULTS: On analyzing 37 adult patients, all had residual Philadelphia (Ph) chromosome. Classic Ph fusion pattern was seen in 33 (89%), derivative chromosome 9 [der(9)] abnormalities in 25 (67.5%) and supernumerary Ph chromosomes in 11 (30%) patients. Coexistence of classical fusion and der(9) abnormalities was seen in 21 patients (57%); and classical fusion, der(9) abnormalities and supernumerary Ph chromosome in 8 patients (22%). None of the patients had BCR-ABL1 gene amplification. There was significant difference in the der(9) abnormal cell percentages between patients with e13a2 and e14a2 transcripts (P = 0.008) and patients with disease transformation (P = 0.007). CONCLUSION: A high frequency of der(9) abnormalities and absence of BCR-ABL1 gene amplification was seen in imatinib-resistant CML patients analyzed. The use of TCDF probe for cytogenetic follow-up in CML patients was found to be useful in identifying BCR-ABL1 related aberrations. The identified patterns in this study, can serve as a reference material for I-FISH signal interpretation using TCDF probe.
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The spectrum of red blood cell parameters in chronic kidney disease: A study of 300 cases p. 61
Indira Shastry, Sushma Belurkar
DOI:10.4103/joah.joah_13_19  
AIMS: To analyze various changes in the red blood cell (RBC) parameters in patients with chronic kidney disease (CKD) and to correlate it with the stage of the disease. MATERIALS AND METHODS: The following parameters were analyzed in 300 diagnosed cases of CKD: RBC count; hemoglobin (Hb); hematocrit; mean corpuscular volume (MCV); mean corpuscular hemoglobin (MCH); MCH concentration (MCHC); serum iron profile (serum total iron, total iron-binding capacity, and serum ferritin levels); and peripheral smear, blood urea, and serum creatinine levels. The data were retrieved from the laboratory information system, and SPSS version 20 was used for statistical analysis. RESULTS: The mean age of presentation was 52 ± 14 years. The male-to-female ratio was 4.3:1. The mean RBC count was 3.29 ± 0.79 × 106/μl, and a significant fall was noticed as the stage of CKD progressed. 74% and 60% of patients with Stage 4 and 5 CKD, respectively, showed Hb of <10 g/dl. Correlation of MCV, MCH, and MCHC values with stages of CKD was statistically not significant. One hundred and six (59%) patients had anemia of chronic disease as per the serum iron profile. 94% of patients showed normocytic normochromic RBC picture on peripheral smear and 21% showed features of hemolysis. CONCLUSION: Anemia is a leading cause of morbidity in patients with CKD and it worsens with the stage of the disease. Evaluation of Hb and RBC parameters in patients with CKD helps in classifying the type of anemia and aids in choosing the correct treatment modalities and avoids unnecessary iron overload in these patients.
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CASE REPORTS Top

Desensitization with plasma exchange in a patient with human leukocyte antigen donor-specific antibodies before T-cell-replete haploidentical hematopoietic stem cell transplantation p. 67
Rasika Dhawan Setia, Mitu Dogra, Divya Doval, Sanjeev Kumar Sharma, Dharma Choudhary, Anil Handoo
DOI:10.4103/joah.joah_72_18  
The presence of human leukocyte antigen (HLA) donor-specific antibodies (DSAs) increases the risk of graft failure in T-cell-replete haploidentical hematopoietic stem cell transplantation (haploidentical-HSCT). We report a case of a 20-year-old male with high-risk thalassemia major who received a haploidentical-HSCT from his mother. Pretransplant recipient screening examination showed positive DSA levels against class I and class II HLAs. The patient underwent a desensitization program consisting of plasma exchange and polyvalent intravenous immunoglobulins. This protocol resulted in the disappearance of DSA class I and reduction in Mean Fluorescence Intensity (MFI) class II antibodies. Engraftment was prompt with stable full donor chimerism. This case report suggests that the adopted scheme is safe for reducing DSA levels and facilitating donor engraftment in patients scheduled for haploidentical-HSCT, though it needs evaluation in a large cohort of patients.
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Homozygous prothrombin gene mutation and ischemic cerebrovascular disease: A case report and review of literature p. 70
Mohammed AlSheef, Mohammed S Almohaya, Arshad M Mian, Saad Mohammed Aljuhayyim
DOI:10.4103/joah.joah_17_19  
This report describes a 32-year-old Saudi nonsmoker man who presented to the emergency department with a history of left-sided hemiparesis and no risk factors for cerebral stroke being reported, except type II diabetes mellitus. Magnetic resonance imaging (MRI) showed an ischemic stroke involving the right putamen and the posterior limb of the right internal capsule. During the hospitalization, he developed new weakness bilaterally, and repeat MRI showed infarctions involving both basal ganglia with a new acute infarct in the left putamen. The patient had normal laboratory hematological investigations; however, he was found to be homozygous for the G20210A mutation in the 3'-untranslated region of the prothrombin gene. In the absence of multiple risk factors that are usually related with cerebral stroke, the association between homozygous prothrombin gene mutation and cerebral stroke was suggested.
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IMAGE IN HEMATOLOGY Top

Metastatic malignant melanoma in the bone marrow with occult primary p. 73
Mansoor C Abdulla
DOI:10.4103/joah.joah_14_19  
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LETTER TO THE EDITOR Top

Persistent diarrhea, hemolytic anemia, and splenohepatomegaly due to vitamin b12 deficiency in an infant p. 75
Mahmood Dhahir Al-Mendalawi
DOI:10.4103/joah.joah_24_19  
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