Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

Background & objectives: Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan. Methods: In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones. Blood samples were processed using fluorescein-labelled proaerolysin, CD14, CD24, CD33 and CD45 panels for granulocytes and monocytes and CD59 and CD235a panel for red blood cells. Results: Three cases (4.92%) having large clones on monocytes as well as granulocytes, which fulfilled the diagnostic criteria of PNH were detected. Further, three cases (4.92%) showed small clones on both granulocytes and monocytes. Nine (15%) cases showed small clones only on granulocytes, and 11 (18%) cases showed small clones only on monocytes. Interpretation & conclusions: The results of the present study suggest that a higher proportion of patients had PNH in western Rajasthan compared to previously reported studies from elsewhere. It is suggested that PNH testing should be added to the procoagulant work-up panel in institutions of this region where it is not routinely done. This provides an otherwise missed opportunity to diagnose this disorder. Eculizumab may be employed, which is effective in reducing thrombophilic events in cases of PNH

Electrolyte changes in the neonates receiving phototherapy

Background: Neonatal jaundice is the most commonly observed clinical condition of early neonatal period. It remains a common cause of readmission and important cause of concern for the parents and clinicians. Therefore, appropriate management of neonatal hyperbilirubinemia is of paramount importance. Phototherapy plays a significant role in its management but this modality is not devoid of complications. Authors studied electrolyte changes in the neonates, who received phototherapy.Methods: This perspective hospital based observational study conducted over the period of one year on 90 eligible admitted neonates who received phototherapy as per AAP guideline. Serum bilirubin, sodium, potassium and calcium were determined before and after termination of phototherapy. The first samples were considered as controls and compared with the samples collected after termination of phototherapy.Results: The incidence of LBW babies in this study was 21.9%, while mean birth weight and gestational age was2.76±0.38 kg and 38.34±0.88 weeks respectively. Mean duration of phototherapy was 38.48±09.34 hours. The mean sodium, potassium and calcium level before therapy were 146.6±6.2 mg/dl, 4.7±0.47 mg/dl and 9.4±0.73 mg/dl respectively. Authors found statistically significant changes in the electrolyte’s levels (Na+ 141.3±6.1, K+ 4.2±0.51 and Ca+2 8.4±0.68 respectively), after phototherapy however none of any neonate shown any clinical signs of dyselectolytemia.Conclusions: Neonates undergoing phototherapy are at greater risk of dyselectrolytemia especially in preterm babies therefore close clinical and biochemical monitoring is required for the prompt management of any significant electrolyte imbalance.

Volume, conductivity, and scatter parameters as diagnostic aid to bacterial sepsis: A tertiary care experience.

Introduction and Materials and Methods: Early diagnosis of sepsis is extremely important to reduce high mortality and morbidity. In this study, clinical usefulness of the volume, conductivity and scatter parameters (mean channels of cell volume, conductivity, and light scatter) in neutrophils was analyzed for predicting acute bacterial infection, which are obtained by the Coulter LH 750 Hematology Analyzer (Beckman Coulter, Fullerton, CA, USA) during automated differential counts. Results: Peripheral blood samples from 162 patients with positive blood cultures for bacteria and 40 healthy controls were studied. We observed a significant increase in the mean channel of neutrophil volume (MNV) from septic patients compared with control subjects (156 ± 13.5 vs. 143 ± 4.8; P < 001). Discussion and Conclusion: An elevation of the MNV was associated with a higher white blood cell count and percentage of neutrophils and was present even in patients who did not have leukocytosis or neutrophilia. With a cut-off of 149 for the MNV, a specificity of 91.4% and sensitivity of 88.7% were achieved. As a quantitative, objective, and more sensitive parameter, we propose that the MNV has a potential to be an additional indicator for acute bacterial infection.

A 60-year-old lady with leonine facies: A rare diagnosis.

Systemic mastocytosis is a rare clonal disorder characterized by mast cell infiltration of one or more organs, with or without skin involvement. Leonine facies is a rare presentation and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face as a result of long standing disease. Bone marrow aspiration and biopsy findings in a 60-year-old woman, who had extensive cutaneous infiltration due to systemic mastocytosis, resulting in 'leonine facies,' are described, and causes of leonine facies are discussed.