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Haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially aggressive and life-threatening syndrome of overactive histiocytes and lymphocytes that commonly affects infants; it is also observed in children and adults of all ages. The disease is differentiated into either primary or secondary causes. Primary HLH tends to be of genetic origin, while secondary HLH results from either infection, autoimmune disorders, or malignancies. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. This paper presents a case of HLH in a tertiary care hospital, associated with adult-onset Still’s disease, diagnosed on both biochemical criteria and histopathologic examination of bone marrow smear.

A 30-year-old Asian male with a significant history of deep vein thrombosis and family history positive for pulmonary embolism presented with complaints of fever, nonradiating epigastric pain, and a sense of abdominal fullness. After the initial workup, ultrasonography of the whole abdomen was carried out which showed thrombus formation in the portal vein. A CT scan of the abdomen was performed, which showed findings suggestive of mesenteric panniculitis. Keeping the significant family history and imaging findings in mind, the clotting and thrombin profiles were analysed and came back positive for the factor V Leiden gene (homozygous). A CT angiogram was performed to demonstrate extensive thrombosis throughout the abdominal vasculature with cavernous transformation. It is asserted that the chronic thrombosis on a background of factor V mutation led towards chronic inflammation of the mesentery. To the authors’ knowledge it is the first reported case of mesenteric panniculitis in a patient with factor V homozygous gene mutation.

Myelofibrosis is characterized by a group of clonal neoplastic proliferation under the influence of cytokines such as fibroblast growth factor (particularly by megakaryocytes) which leads to the replacement of normal hematopoietic bone marrow with connective tissue via collagen fibrosis. It most commonly affects patients in the fifth and sixth decade of their life. Constitutional symptoms such as fatigue along with massive splenomegaly, easy fatigability, and easy bruising are reported most commonly in such patients. Here we present a case of a 76-year-old male who was diagnosed with myelofibrosis having transient pancytopenia and CD-68 marker positive.