A Rare Case of Hemophagocytic Lymphohistiocytosis (HLH) with Protein Energy Malnutrition (PEM) Treated in KIMS Pediatrics Department
Hemophagocytic Lymphohistiocytosis (HLH) is a rare but fatal hematological disorder associated with impaired natural killer and cytotoxic T cell function. Transformed macrophages known as hemophagocytic histiocytes can invade bone marrow, spleen, lymph nodes, liver, and skin leading to multisystem organ failure. The clinical manifestations include prolonged fever, visceromegaly, cytopenias, low fibrinogen level, raised triglyceride, ferritin and soluble IL-2 receptor levels, histological evidence of benign hemophagocytic macrophage and impaired NK cell activity on flow cytometry. The overall mortality has been documented to exceed 50% and most of the patients have been reported to die within the first 2 months of treatment. HLH can be characterized into primary and secondary diseases based on underlying etiology. Primary HLH is also acknowledged as Familial Hemophagocytic Lymphohistiocytosis (FHL), which presents as an autosomal recessive disorder in the pediatric age group. It is mainly associated with defects in genes involved in cytolytic activity of cytotoxic NK cells and cytotoxic T lymphocytes. Secondary or acquired HLH may be due to infections, autoimmunity, auto-inflammatory disorders and malignancies. Some immunodeficiency syndromes and hereditary diseases have been proved to be associated with HLH, e.g., Chediak Higashi syndrome, Wiskott’s Aldrich syndrome and X linked lymphoproliferative syndrome.
A one-year old female baby A. Sahoo, was presented with complaints of intermittent fever for one month, abdomen fullness for one month and bilateral pedal edema for 4 days and Red spots on right foot for the last 1 day. Though she had a history of blood transfusion 2 days back, there was no history of any chronic illness. The Baby was delivered at home, weighing 2.4kg at birth and cried immediately after birth. She had a 6 year old elder sister doing well.
On examination, she had pallor ++, bilateral pitting edema, enlarged cervical lymph node, distended abdomen with hepatosplenomegaly, and anthropometry revealed protein energy malnutrition. Biochemical investigation revealed Vit B12, folic acid, and iron deficiency anemia. S.Triglyceride-131 mg/dL and S.Ferritin-31.4 ng/mL, normal USG with mild ascites. MP ICT, Typhidot, and Scrub typhus were negative. HPLC showed sickle cell trait. Anti SARS-CoV-2 level was 1.55, ESR-48 mm in 1st hour, ANA-1:80, S.Fibrinogen-249 mg/dL, blood and urine c/s, mantoux test, stool occult blood, coomb’s direct test and TORCH panel were negative. Bone marrow biopsy showed mild erythroid hyperplasia with hemophagocytosis. A diagnosis of PEM with HLH was made.
The Child was started on the F 100 diet, Dexolac HIKAL on alternate feeds, supplements of vitamin B12 & folic acid were started. Specific treatment with omnacortil was started. The kid recovered well and got discharged.
Parents were very happy to get their child diagnosed and treated properly in KIMS. They bestowed their blessing to staff and doctors taking care of their kid. The Pediatric Team of KIMS is thankful to the management for providing infrastructure and diagnostic facility to carry-out its work.
This case was managed by Prof. M.R. Behera; Prof. S.R. Biswal; Prof. Nirmal K. Mohakud, Dr. P. Dash and Post graduates (Dr. Ramkumar, Dr. Praneeth, Dr. Jenith, Dr. Puramjay) and the whole pediatric team.
Summer Training Programme on “Industrial Automation PLC”
The summer training programme on ‘Industrial Automation PLC’ at School of Electrical Engineering, KIIT-DU concluded after a rigorous one month schedule. The programme had commenced on 1st June, 2021. Prof. Subrat Behera, Associate Dean-T&P, SoEE and FIC Training Program, School of Electrical Engineering was associated with the entire training program. It was successfully conducted by Mr. Md Aftab Alam, expert trainer of PLC, SoEE with the active involvement of Mr. Nabakishore Dash, Mr. K. C. Barik, Mr. Sibananda Sahoo and Mrs. Bandana Bahel.
Dr. Byamakesh Nayak, Dean of the School guided the programme as per current industrial requirements. The new batch of regular training programme on “Industrial Automation PLC” is going to start from 1st August, 2021. The entire programme was well coordinated by Ms. Susree Subinita, AAO, School of Electrical Engineering.
A Programmable Logic Controller (PLC) is an industrial system that has been well accepted and adapted for the control of manufacturing processes such as assembly lines, machines, robotics devices. It is significantly used in activity that requires high reliability, ease of programming, and process fault diagnosis. It has been widely used in automation industries, power plants, airports, process plants, packaging industries and many more.