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   Table of Contents - Current issue
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January-March 2021
Volume 12 | Issue 1
Page Nos. 1-57

Online since Monday, March 15, 2021

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ORIGINAL ARTICLES  

Analysis on the composition of leukapheresis product – A comparison between MCS+® + and spectra optia® apheresis equipment p. 1
Mohandoss Murugesan, Gayathiri K Chellaiya, Chandran K Nair, Sangeetha K Nayanar
DOI:10.4103/joah.JOAH_97_20  
INTRODUCTION: Peripheral blood stem cell has become the preferred source for hematopoietic stem cells in both autologous and allogenic transplants. CD34+ cells represent a small proportion of the leukapheresis product content. The present study aims to analyze the cellular composition of leukapheresis product collected by intermittent-flow Mobile Collection System®+ (MCS+) and continuous-flow (Spectra Optia®) apheresis equipment. METHODS: In this retrospective study, 97 leukapheresis procedures for 85 individuals mobilized only with granulocyte colony-stimulating factor were analyzed. The product samples were analyzed for CD34+ cells, red blood cells (RBCs), white blood cells, and platelet contents. The difference in overall product composition was compared between the equipment through Mann–Whitney U-test. RESULTS: Both the equipment had similar CD34 and mononuclear cells (MNC) harvested in the product. However, Spectra Optia had statistically significantly lower product volume than MCS+ (215 mL vs. 260 mL, P = 0.04). Similarly non-CD34 composition such as RBC content per apheresis was six-fold higher (27 mL vs. 4 mL) and platelet contamination (1601 vs. 1275 × 109/L) was relatively higher with MCS+ over Spectra Optia. No relationship was observed between the CD34 concentration in the product and RBC and platelet contamination between both the equipment. CONCLUSION: Both equipment collect adequate CD34 and MNC cells; however, Spectra Optia is preferred due to product quality in terms of less product volume with minimal RBC and platelet contamination.
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Patterns of blood products utilization at a tertiary care center in the Southern Region of Saudi Arabia p. 6
Husain Y Alkhaldy, Bushra Saeed AlShahrani, Ali M. Alkhaldi, Abdullah Salman A. Alqahtani, Ibrahim Muhayya, Mohammed Alqahtani, Mohamed Eissa
DOI:10.4103/joah.joah_112_20  
MATERIALS AND METHODS: A total of 457 blood transfusion requests for 244 patients were received over a 3-month period. Registered demographic characteristics (age, sex, and nationality), ward, type of blood, and hemoglobin concentration before transfusion for all the patients were collected. RESULTS: A total of 1328 blood products were requested, while 780 (59%) units were transfused. The cross-matched packed red cells/transfusion ratio was 1.8, while request/transfusion was 1.7 for the fresh frozen plasma and 1.23 for platelets. Packed red blood cells were the main blood product used in blood transfusion (BT). General surgery/orthopedics and intensive care unit were reported with a greater number of requests. The main indications noted for blood requisition were surgery and anemia. CONCLUSIONS: Current guidelines appear to be respected within services regarding BT decision criteria. However, about half of the requested blood is not transfused which suggest further improvement. Adherence to established guidelines seems variables among different departments. Elective transfusion medicine rotations for all trainees and specialized dedicated sessions for doctors may help in further betterment of utilization of blood bank services.
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Rates of bleeding, thrombosis, and survival in patients who underwent hematopoietic cell transplantation at king abdulaziz medical city, Riyadh, Saudi Arabia p. 10
Sultan Alqahtani, Ahmed Alragea, Basil Alqahtani, Nawaf Alhoshan, Ibrahim Aljasser, Abdulaziz Alobaied, Mohammed Alassiri, Emad Masuadi, Mohsen Alzahrani
DOI:10.4103/joah.joah_120_20  
BACKGROUND: Bleeding tendency and thrombosis are two major hematological complications observed in patients after hematopoietic cell transplantation (HCT). Although these complications are well reported in western communities, they are not well established in Saudi Arabia. OBJECTIVES: This retrospective study investigated the rates of bleeding, thrombosis, and survival in Saudi and non-Saudi patients who underwent HCT at King Abdulaziz Medical City, Riyadh, Saudi Arabia, from 2010 to 2017. METHODS AND MATERIALS: A total of 372 Saudi and non-Saudi patients of both sexes with leukemia, anemia disorders, lymphoma, and other types of pathological disorders who underwent autologous or allogeneic HCT were included in this study. Patient data including age, sex, nationality, type of cancer, transplant type, coagulation profile, date of operation and discharge, treatment, and other outpatient notes were collected and analyzed using the Chi-square test. RESULTS: The majority of our patients undergoing allogeneic transplantation had leukemia and other types of anemia disorders including aplastic anemia, sickle cell anemia, and β-thalassemia (79%). However, the majority of patients undergoing autologous transplantation (73.4%) had lymphoma and other types of pathological disorders, including liver cell carcinoma, nephroblastoma, neuroblastoma, myelodysplastic syndromes, and primitive neuroectodermal tumors. Among patients with leukemia, anemia disorders, lymphoma, and other pathological disorders, 2.2%, 1.4%, 1.4%, and 2.8% suffered from thrombosis (overall, 1.9%) and 9.5%, 8.6%, 5.6%, and 11.2% suffered from bleeding, respectively (overall 8.9%). Moreover, the 7-year survival rate among those patients was 82.5%. CONCLUSION: Bleeding occurred at a higher rate after HCT, and the overall 7-year survival rate was relatively high.
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Ferric carboxymaltose solution versus iron sucrose complex in treating Iron-deficiency anemia patients with heavy uterine bleeding: A cost-efficacy study from a tertiary care hospital in the Kingdom of Saudi Arabia Highly accessed article p. 17
Ayman Hejazi, Omneya Mohamed, Mansour Alhowimel, Maysoun AlAdham
DOI:10.4103/joah.joah_134_20  
BACKGROUND: Heavy uterine bleeding (HUB) affects 4.0%–51.6% of women and is responsible for the high prevalence of iron-deficiency anemia (IDA). Ferric carboxymaltose (FCM) is a novel Type I polynuclear iron (III)-hydroxide carbohydrate complex that is highly stable and requires short administration time. The current study estimated the budgetary impact of adopting FCM to treat IDA in HUB patients from the perspective of a tertiary care hospital in the Kingdom of Saudi Arabia (KSA). SUBJECTS AND METHODS: A budget impact model was adopted to compare the total annual costs of iron sucrose complex (ISC) versus FCM from a tertiary care hospital setting perspective in a 1-year time horizon. RESULTS: The total annual cost in ”ISC” scenario was higher than in ”FCM” scenario (Saudi Riyal [SAR] 1,079,535 vs. SAR 724,981), resulting in a cost saving of SAR 355,000 over a 1-year time horizon with FCM. Lower expenditure on health professionals and lower costs of disposables and overhead were the main drivers to the cost savings accounting for nearly 96% of the savings. Although the direct IV iron cost was higher in ”FCM” scenario, the increase in pharmaceutical drug cost was offset by the savings in the cost associated with disposables (−SAR 232,000) and resource utilization (−SAR 451,195). CONCLUSION: FCM was associated with cost savings as compared to ISC for the treatment of IDA in HUB patients from the tertiary care hospital perspective in the KSA.
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Caregivers' Perception of iron overload and its implications p. 22
Nof Saadi Alqarni, Ali H Algiraigri
DOI:10.4103/joah.joah_144_20  
BACKGROUND: Although frequently diagnosed in our region, transfusion-dependent thalassemia (TDT) often remains a poorly managed disease. This is due to a lack of awareness of the long-term effect of iron overload among thalassemic patients. This study aimed to examine the unmet needs of TDT patients and provide insight into achieving better care. METHODS: We conducted a cross-sectional study of 50 caregivers of heavily transfused thalassemia and sickle cell anemia (SCA) patients. The participants were recruited during their visit to the pediatric hematology clinic or daycare unit between September 2018 and May 2019. The researchers administered a questionnaire to assess the awareness of iron overload and compliance with its management in TDT and SCA. RESULTS: Most participant caregivers of children with TDT and SCA had a low level of awareness about the most critical complication of TDT and SCA, which is iron overload. Furthermore, two-thirds of the participants did not appreciate the seriousness of iron overload nor its potential complications. Moreover, the participants reported that their health-care providers rarely or occasionally discuss iron overload or its management. Furthermore, they reported that compliance with health-care appointments and iron chelation therapy was suboptimal. CONCLUSION: Caregivers of TDT and SCA patients reported a low level of knowledge of the magnitude of iron overload, its management, and expected complications if not treated appropriately. Health education on iron overload is critical to ensure appropriate compliance and consequently, control of such disease. The focus must be shifted from giving blood to eliminating excess iron among TDT patients.
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Study of coagulation profile in patients admitted to a trauma center in a tertiary care hospital p. 26
Kalpana Ketan Kulkarni, Anup Pravin Bhandari, Pallavi Rathod
DOI:10.4103/joah.joah_151_20  
CONTEXT: Globally, trauma is the leading cause of death in young adult population, with approximately accounting for 10% of all deaths. Trauma-induced coagulopathy (TIC) is an independent predictor of morbidity and mortality. AIMS: The aim of the study was to find the prevalence of abnormal test results of coagulation on admission to a trauma center and correlate them with mortality. SETTINGS AND DESIGN: This was a cross-sectional observational study conducted on patients admitted to a trauma center of a tertiary care hospital. SUBJECTS AND METHODS: A total of 149 patients with an Injury Severity Score >15 were included in the study. Blood samples of the patients were collected within 24 h of trauma and 2 h of admission and tested for platelet (PLT) count, prothrombin time (PT), activated partial thromboplastin time (APTT), and fibrinogen (FIB) level. The patients were stratified into two groups – presence/absence of TIC and followed for 2 weeks for mortality. STATISTICAL ANALYSIS USED: Data analysis was done using Microsoft Excel and statistics software SPSS 20 version. RESULTS: Among the 149 patients, 84 had TIC, out of which 71.43% of the patients showed mortality. Of the remaining patients who had no TIC, 35.38% reported mortality. If all the four parameters, that is, PT, APTT, FIB, and PLTs, were considered together, then the percentage of mortality increased to 89.47%. If PT and APTT were studied together, then the percentage of mortality increased to 85.71%. If PT, APTT, and FIB were studied together, the mortality was 84.62%, whereas for parameters PT, APTT, and PLTs together, the mortality was 80%. CONCLUSIONS: Coagulopathy is a strong predictor of mortality in major trauma patients. Basic coagulation tests appear to be sensitive tools for identifying patients at high risk of early death and can be used for early minute diagnosis of TIC. Patients with TIC should be managed aggressively to prevent mortality.
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Simple predictors of peripheral blood stem cell yield in healthy donors: A retrospective analysis in a tertiary care hospital p. 31
Thulasi Raman Ramalingam, Lakshman Vaidhyanathan, Anurekha Muthu, Vikram Prabhakar, Balasubramaniam Ramakrishnan, Revathi Raj, Jose M Easow
DOI:10.4103/joah.joah_100_20  
BACKGROUND: Peripheral blood stem cells (PBSCs) are commonly used for hematopoietic stem cell transplant (HSCT) over other stem cell sources. The hematopoietic stem cells (HSCs) are mobilized from marrow by granulocyte colony-stimulating factor (G-CSF) and then harvested by apheresis technique. The HSC yield differs in donors that may be due to inadequate mobilization or difficulty in harvesting the mobilized stem cells. MATERIALS AND METHODS: We retrospectively analyzed donor demographic and pre-apheresis hematological variables with circulating CD34+ cell (cir CD34) count and HSC yield in product in 100 normal donors. G-CSF was given for 5 consecutive days, and the stem cells were harvested on day 5. The cir CD34 count and pre-apheresis variables were recorded a day before harvest. RESULTS: Of 100 donors, 77% were males and 23% were females. Male sex, younger age, and donor weight were significantly associated with better CD34 yield in the product. Among the pre-apheresis hematological variables, absolute neutrophil count, hematocrit, and absolute nucleated red blood cell count significantly correlated with post-GCF circulating CD34 and CD34 yield in the product. Donors with mean corpuscular volume <80 fL showed relatively poor CD34 cell harvest as compared to normal donors, though with adequate mobilization. CONCLUSION: Selection of donors for PBSC apheresis is crucial for a good transplant outcome and recovery. Alternate strategies that improve the final CD34 yield should be employed for donors with high risk for poor CD34+ cell yield.
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CASE REPORTS Top

Primary osseous burkitt lymphoma mimicking multiple myeloma – A case report and review of literature p. 37
Somnath Roy, Tanmoy Kumar Mandal, Lingaraj Nayak, Avinash Gupta, Dhanlaxmi Lalit Shetty
DOI:10.4103/joah.joah_107_20  
Burkitt lymphoma (BL) is one of the aggressive subtypes of non-Hodgkin lymphoma commonly seen among pediatric and young population. Primary BL of pelvic bone presenting with back pain, paraparesis, and multifocal osteolytic lesion in the elderly is a rare entity. We report a case of primary BL of pelvic bone in a 65-year-old female presenting with back pain and paraparesis, facing diagnostic dilemma as the lesions were radiologically mimicking multiple myeloma. Subsequently, bone marrow flowcytometry and cytogenetic evaluation by fluorescence insitu hybridization confirmed the diagnosis. We highlight the diagnostic challenge, clinical presentation, and treatment strategy in such a rare case along with a review of literature.
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A rare presentation of childhood acute lymphoblastic leukemia with hypereosinophilia lacking peripheral blood smear leukemic cells p. 41
Ahmed I Yakoub
DOI:10.4103/joah.joah_108_20  
Presentation of childhood acute lymphoblastic leukemia (ALL) with hypereosinophilia is rare and may be confusing, especially in the absence of blasts on examination of the peripheral blood film. It is usually associated with cytogenetic abnormalities. Hypereosinophilic childhood ALL has a poor prognosis requiring prompt clinical intervention. A case of 10-year-old boy presented to our hospital with a high fever not responding to treatment and hypereosinophilia in the peripheral blood, diagnosed later as childhood ALL.
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Intravenous Iron sucrose induced bullous pemphigoid: A rare case report p. 44
Kaustav Saha, Shatavisa Mukherjee
DOI:10.4103/joah.joah_121_20  
Pemphigus is an autoimmune bullous disease that may be influenced by multitude of genetic and exogenous factors. Drug induced pemphigus also holds a center stage. With culprit molecule varying from penicillamine to various thiol associated drugs, management mainly revolves around proper history elicitation and prompt withdrawal of suspected drug. The present case describes a rare presentation of intravenous iron sucrose induced bullous pemphigoid.
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Cutaneous manifestation of sickle cell disease p. 46
Rashmi Patnayak, Prateek Das, Sukanya Patra, Rajesh Kumar Bhola
DOI:10.4103/joah.joah_123_20  
Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy. SCD patients commonly present with skin ulceration. This case is about a 22-year-old male whose initial presentation was reddish spots on both lower legs. Skin biopsy revealed features of leukocytoclastic vasculitis with sickled red blood cells (RBCs). He was further evaluated with sickling test which was positive. His hemoglobin electrophoresis was reported as sickle-beta thalassemia. This case is presented to emphasize the importance of RBC morphology and high index of suspicion, particularly in dealing with patients from the sickle cell belt area.
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Osteoblasts to the Rescue – A Rare Case of Secondary Myelofibrosis p. 48
Lawanya Gunaseelan, Eliz Thomas, Prasanna N Kumar, Sujaya Menon
DOI:10.4103/joah.joah_126_20  
A 36-year-old female patient presented with joint pain, generalized weakness, and bleeding gums. There was no significant history of other illnesses or intake of drugs. Examination revealed pallor and splenomegaly. Peripheral smear examination showed pancytopenia. A large number of osteoblasts and a few scattered osteoclasts were seen in the bone marrow aspirate. The trephine biopsy revealed marrow fibrosis. A suggestion to investigate for hyperparathyroidism was made. Serum calcium, ionized calcium, and PTH were increased. 99m Tc-Sestamibi scan was done and adenoma was detected in the right inferior parathyroid and subsequently excised. Postsurgery, hematological parameters and bone markers improved. Follow-up bone marrow biopsy revealed hypercellular marrow with marked regression of fibrosis. Splenomegaly was reversed. We concluded that pancytopenia was due to bone marrow fibrosis, resulting from primary hyperparathyroidism. It is important to consider secondary causes of myelofibrosis in the appropriate clinical setting.
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Granulocytic sarcoma: A case series and review p. 51
M .P Rakesh, Nizar Mullali Mohamed Kunhi, Niranjan Vijayaraghavan, KV S. Latha
DOI:10.4103/joah.joah_133_20  
Granulocytic sarcomas (GSs) are rare, solid, extramedullary tumors composed of immature myeloid cells. They can be seen before, concomitantly with or after a diagnosis of acute myeloid leukemia, chronic myeloid leukemia, or myelodysplastic syndromes. We report four unusual presentations of granulocytic sarcoma treated at our center. Due to the rarity of the disease, it is difficult to conduct randomized controlled trials, and hence, there are no consensus for the treatment of GS. Most of the isolated GS frequently progress to acute myeloid leukemia, and all GSs tend to have a poor prognosis.
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IMAGES IN HEMATOLOGY Top

Signet ring follicular lymphoma - A rare entity p. 55
Manu Goyal, Ayeesha Shaikh Begum
DOI:10.4103/joah.joah_66_20  
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Endothelial cells in the peripheral blood smear: An incidental finding p. 57
Shreyam Acharya, Abhirup Sarkar, Aparna Ningombam
DOI:10.4103/joah.joah_131_20  
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