Cytophagic histiocytic panniculitis associated with HBe hemoglobinopathy in a patient with hemophagocytic syndrome

Abstract

Introduction: Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis which may occur alone or as a part of systemic manifestation of Hemophagocytic syndrome (HPS). It is described as a chronic histiocytic disorder of the subcutaneous adipose tissue with lymphocytic and histiocytic infiltration showing hemophagocytosis. It may also be noted in bone marrow, spleen, lymph nodes and liver. Treatment includes glucocorticoids, cyclosporine and combined chemotherapeutic medications. Observation: A 34 years old lady, presented with multiple nodules over the body since 2 years. Hematological investigations revealed that patient had a rare HbE hemoglobinopathy and was treated for that. Skin biopsy showed CHP and subsequently on hematological and biochemical tests, a diagnosis of HPS was given and patient was referred to a hemato-oncologist. Conclusion: Cytophagic histiocytic panniculitis is a rare and fatal form of panniculitis with multisystem involvement. Awareness of this cutaneous manifestation may help physicians in the early diagnosis of HPS. We report this interesting case of CHP with a brief review of literature. To best of our knowledge this is the first case of Hemophagocytic syndrome associated with HbE hemoglobinopathy.

comment: Cytophagic histiocytic panniculitis associated with HBe hemoglobinopathy in a patient with hemophagocytic syndrome

“…Cytopenia -Counts below the specified range in at least 2 of the following cell lineages: Absolute neutrophils less than 1000/µL Platelets less than 100,000/µL Hemoglobin less than 9.0 g/dL 4. Hypofibrinogenemia or hypertriglyceridemia - [1] Fibrinogen less than 1.5 g/L or levels greater than 3 standard deviations below the age adjusted reference range value or [2] fasting triglycerides greater than 2 mmol/L or levels greater than 3 standard deviations above the age-adjusted reference range value 5.Hemophagocytosis -Must have tissue demonstration from lymph node, spleen, or bone marrow without evidence of malignancy.…”