Acute Graft versus Host Disease in Beta Thalassemia Patients Following Allogeneic Haematopoietic Stem Cell Transplantation.

OBJECTIVE: To analyse the frequency, risk factors, and clinical symptoms of acute graft-versus-host disease (aGvHD) in patients with beta-thalassemia major after allogeneic haematopoietic stem cell transplantation (HSCT). STUDY DESIGN: Descriptive study. Place and Duration of the Study: Department of Clinical Haematology, Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan, from January 2017 to December 2021. METHODOLOGY: Data were obtained from patients diagnosed with bone and tissue malignancies (BTM) who had undergone haematopoietic stem cell transplantation (HSCT) and experienced aGVHD. Patients who experienced initial graft failure and individuals who underwent subsequent bone marrow transplantation were excluded. RESULTS: Total of 117 patients diagnosed with BTM underwent fully matched HSCT, including 76 (65%) males, and 41 (35%) females. The median age of the patients undergoing transplantation was 7.34±7.32 years and the donors' median age was 7.6±9.85 years. Among the donors, 53 (45.3%) were males and 64 (54.7%) were females. Gender disparity was observed in 46 (39.3%) instances as a female donor matched with a male recipient. A total of 106 individuals underwent bone marrow harvest (BMH); with 5 (4.3%) patients receiving peripheral blood stem cells (PBSC) and 6 (5.2%) patients receiving both BMH and PBSC. Acute GvHD was observed in 50 (42.7%) patients, including 30 (60%) males and 20 (40%) females. Grade I GvHD occurred in 32 (27.3%) individuals, Grade II GvHD in 16 (13.7%) patients, and Grade III GvHD in one (0.8%) patient. It had no statistically significant association with recipient/donor age, gender disparity, the source of the graft source, the dose of stem cells, or the presence of thymoglobulin (TG). CONCLUSION: Acute GvHD was observed in high frequency in Beta-thalassemia patients receiving morrow harvesting proportional to their gender distribution. Associated factors were GvHD prophylaxis measure, mucositis and, CMV reactivation. KEY WORDS: Beta thalassemia major patients, Acute graft versus host disease, Allogeneic haematopoietic stem cell.

Diagnostic Accuracy of Immature Platelet Fraction (IPF) to Differentiate Between Thrombocytopenia due to Peripheral Destruction versus Bone Marrow Failure.

OBJECTIVE: To analyse the predictive value of immature platelet fraction (IPF) as an independent diagnostic marker to differentiate between hyperdestructive and hypoproductive thrombocytopenia. STUDY DESIGN: Cross-sectional observational study. Place and Duration of the Study: Armed Forces Institute of Pathology Rawalpindi, from February to July 2022. METHODOLOGY: A total of 164 samples were included in the study by non-probability consecutive sampling. Among these, 80 were obtained from normal individuals serving as control; 43 were obtained from patients having hyperdestructive thrombocytopenia (idiopathic thrombocytopenia, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation); and 41 were obtained from those hypoproductive thrombocytopenia (acute leukaemia, aplastic anaemia, chemotherapy). Sysmex automated haematology analyzer, XN-3000 was used to determine the immature platelet fraction (IPF) of the patients. ROC curves analysis was done to ascertain area under curve. RESULTS: Immature platelet fraction (IPF %) was significantly higher in consumptive / hyperdestructive thrombocytopenia group i.e. median (IQR), 21% (14.4-26.2) as compared to 6.5% (4.6-8.9) in hypoproductive thrombocytopenia, and 2.6% (1.3-4.1) in normal control group (p <0.001). Cut-off value with the highest sensitivity and specificity for IPF vs. normal population was 7.95% with sensitivity of 97.7% and specificity of 86%. CONCLUSION: Immature platelet fraction (IPF of 7.95%) possesses high diagnostic accuracy, sensitivity and specificity for differentiation between hyperdestructive vs. hypoproductive thrombocytopenia. It can be used as a reliable marker to differentiate between the two entities. KEY WORDS: Immature platelet fraction, Thrombocytopenia, Bone marrow failure, Peripheral destruction.

Laboratory Evaluation and Pathological Features of Bone Marrow Metastasis in Non-haematological Malignancies.

This study aimed to analyse the diagnostic accuracy of different laboratory parameters that can predict bone marrow metastasis. A cross-sectional analytical study was conducted at the Armed Forces Institute of Pathology (AFIP), Rawalpindi from March 2021 to August 2021. Bone marrow aspirates and biopsy procedures were done on 60 newly diagnosed cases of non-haematological malignancies as part of staging. Laboratory parameters noted for the study included peripheral blood smear findings, serum lactate dehydrogenase (LDH), radiological findings, and bone marrow aspirate/trephine biopsy results. Bone marrow metastasis was seen in 21/60 patients. The most common malignancies with bone marrow involvement were retinoblastoma and neuroblastoma. Laboratory findings showed no significant statistical difference in mean haemoglobin and total leukocyte count between cases and controls. Positive cases had a mean platelet count of 261.7 x 109/L and mean LDH of 750.1 U/L (p <0.05) for both parameters. ROC analysis showed the area under the curve (AUC) for LDH to be 0.969 (highly significant) showing a strong predictive value of LDH. Positive radiological findings were detected in only one case with bone marrow metastasis. The elevated level of serum LDH is not only cost-effective but also has high diagnostic accuracy to predict bone marrow metastasis. Key Words: Bone marrow, Biochemical, Lactate dehydrogenase, Metastasis, Non-haematological malignancies.