RESUMO
Background: Intravenous immunoglobulin (IVIG) replacement therapy is increasingly in demand. This study focused on the characteristics of IVIG usage and associated factors toward the frequency status of IVIG among patients in Hospital Kuala Lumpur. Methods: A retrospective cross-sectional study was performed on patients who received IVIG in Hospital Kuala Lumpur. Data were extracted from the request forms for IVIG recorded in the Pharmacy Department from January 2018 until December 2019. Chi-squared test and t-test analysis were used for statistical analysis, and a P-value of < 0.05 was considered significant. Results: A total of 482 patients received IVIG in Hospital Kuala Lumpur. There were 243 (50.4%) females and 228 (47.3%) males with median age of the patients was 27 years old. The highest indications for IVIG among all patients were hypogammaglobulinemia and other deficiency states in 127 patients (26.3%). The most common indication for one-off treatment in adults was hypogammaglobulinemia and other deficiency states, 35%; whereas in paediatrics, it was Kawasaki disease, 20.3%. The highest indication for regular therapy among adult patients was chronic inflammatory demyelinating polyneuropathy (23.4%), while in paediatrics it was sepsis (31.1%). The clinical category was associated with the frequency status of IVIG usage in both adult and paediatric cohorts with P = 0.004 and P = 0.017, respectively. Conclusion: There were significant differences between the indication of one-off treatment and regular therapy among adult and paediatric patients. A national guideline on the prescription of IVIG for patients is instantly needed to help clinicians in prescribing IVIG appropriately.
RESUMO
Para-Bombay blood phenotype is a rare blood group with limited cases reported worldwide. This blood group is characterized by the absence of ABH antigen on red blood cells but presence of ABH secretor substances in the body secretion. This rare phenotype is usually misinterpreted as O and may endanger the patient if urgent blood transfusion is required. A mother who was labelled as group O Rh D positive during antenatal follow-up was found to have ABO discrepancy during delivery. The newborn was admitted for extremely premature delivery at 25 weeks. As the baby required transfusion, problem arose during cross matching with the mother's sample. It was found that the mother was group O Rh D positive in forward grouping. However, the reverse grouping showed the presence of reaction (2+) in O cells. The baby was grouped as O Rh D positive. As transfusion was urgently needed due to baby's unstable condition, group O Rh D positive packed cell was found compatible with baby's serum, subsequently transfused. Bombay blood donor was contacted, and the donated blood was sent to the hospital for further management. Further investigations were performed, indicating that the mother is para-Bombay A. Due to recent transfusion to baby, we suggested to repeat baby's blood group after the baby is one year old. Para-Bombay was usually mislabelled as O if the sample was not tested with O cell in reverse grouping. Additional tests may be needed during antenatal follow-up to prevent complications during delivery, which requires emergency blood transfusion.
RESUMO
H-deficient phenotype individuals with absent or weak anti-H activity may remain undetected on standard routine blood grouping. We report a case of a 59-year-old-man presented with symptomatic anaemia secondary to upper gastrointestinal bleed with haemoglobin level of 68 g/L who required two units of packed red blood cells. He was previously grouped as O Rh D positive and had a history of uneventful multiple blood transfusions. His latest pre-transfusion investigations showed ABO discrepancy between forward and reverse blood grouping, pan-agglutination in both antibody screening and identification with negative direct Coombs test and autocontrol. Further testing including anti-H lectin test and saliva secretor study confirmed that the patient blood group was para-Bombay B RhD positive. This case highlights that the para-Bombay phenotype can be mistakenly labelled as "O" if further investigations are not performed.
