Haemophagocytic Lymphohistiocytosis in a Malay infant: Rare, Old and Often Forgotten Disease

Haemophagocytic lymphohistiocytosis (HLH) is a rare disease but potentially life threatening clinical syndrome. It is caused by a multisystemic hyperinflammatory process secondary to severe hypercytokinemia with excessive and uncontrolled activation of the immune response. We report a case of familial HLH with no apparent causes in 6 months-old Malay girl presented with recurrent fever associated with severe anaemia and bleeding tendency requiring extensive treatment but refractory to the treatment which lead to mortality due to neutropenic sepsis indicating of poor prognosis of this disease. This familial type of HLH should be suspected in all children after excluding all the secondary causes with collective laboratory features and requiring extensive management as it associated with high mortality. Bangladesh Journal of Medical Science Vol. 21(1) 2022 Page : 196-200

Haemophagocytic Lymphohistiocytosis (HLH) in Adult with Dengue Infection

Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecific clinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-organ dysfunction with superimposed pneumonia, requiring mechanical ventilation. However, persistent cytopenia despite blood transfusion without evidence of haemorrhage raised the suspicion of HLH. Further blood investigations revealed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia. Bone marrow aspiration showed haemophagocytosis. Patient fulfilled the diagnostic criteria for HLH by HLH-2004 trial. Her HScore is 281, with the probability of having HLH is 99.9%. Patient’s condition improved after administration of intravenous immunoglobulin (IVIG) and intravenous dexamethasone in tapering doses. Early specific treatment of HLH with IVIG and/or corticosteroid is important but diagnosis is usually delayed due to nonspecific clinical findings and laboratory results. High index of suspicion with the aid of diagnostic criteria by HLH-2004 trial and HScore is helpful to recognise this syndrome.

A fatal outcome in a patient with coeliac disease who suspended a strict gluten-free diet: a case report

Coeliac disease (CD) is an autoimmune small bowel disease that occurs in susceptible individuals that develop an immunological reaction to gluten. A strict gluten-free diet (GFD) is the primary treatment for CD. This case report describes a patient with CD recurrence due to a discontinuation of a strict GFD by the patient. After recurrence, the patient developed fever and pancytopaenia, and quickly died of haemophagocytic lymphohistiocytosis (HLH). To the best of our knowledge, this is the first description of a case of CD associated with HLH due to discontinued GFD, which may contribute to improving the awareness of the importance of maintaining a strict GFD and having regular follow-up examinations.

Haemophagocytic lymphohistiocytosis (HLH)-associated stress cardiomyopathy secondary to autoimmune conditions successfully treated with anakinra

We describe two young cases of reactive haemophagocytic lymphohistiocytosis (HLH) with the resultant stress cardiomyopathy in the setting of underlying autoimmune diseases, systemic lupus erythematosus (SLE) and Still’s disease. The initial presentation was similar in both cases with fever, hyperinflammatory response, hypotension (vasoplegia), bicytopenia and hyperferritinemia. Despite standard of care and multiple broad-spectrum antibiotics, both cases remained pyrexic and were ultimately admitted to the intensive therapy unit to treat cardiogenic shock. Echocardiogram of both cases showed low ejection fraction, the cause for which was not found until the final diagnosis of HLH was made. Both cases made a complete clinical and cardiac recovery following the initiation of high-dose glucocorticoids and anakinra.