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Ref Number = PITIKA-ASPR0043
THE UNCONTROLLED GUARDIAN: HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
Jayson Nino Yerro M.D.
Hemophagocytic lymphohistiocytosis is a rare but potentially fatal disease, which primarily affects infants. This is a report of a 1 year old and 2 months female who presented initially with fever, erythematous wheals and cervical lymphadenopathies. This case report was conceptualized to present a case of a rare but potentially life-threatening condition brought about by the under regulation of our immune system, this report aims to enlighten our colleagues regarding this condition.
This is a case of a 1-year-old female, presenting with intermittent fever for 7 days accompanied by pallor, multiple erythematous wheals on the cheek and extremities, multiple lymphadenopathies and splenomegaly. There were no noted malignant diseases in her family. She was initially managed as a case of typhoid fever; further investigation showed anemia, thrombocytopenia and immature cells. A suspicion of leukemia was considered at this point, bone marrow smear microscopy was done showing no significant blast population. Another consideration was histiocytosis, further ancillary procedures were done such as serum ferritin, lipid profile, these ancillary tests revealed hypertriglyceridemia, hyperferritinemia. These results pointed to the diagnosis of hemophagocytic lymphohistiocytosis by fulfilling five of the eight signs or symptoms, fever, splenomegaly, thrombocytopenia, anemia, hypertriglyceridemia and hyperferritinemia.
hemophagocytic lymphohistiocytosis is characterized as a severe immunodeficiency brought about by the uncontrolled immunoregulatory function of the cytotoxic T cells, macrophages and natural killer cells causing a cycle of uninhibited cytokine release. This condition often presents with fever, rashes, weight loss, lymphadenopathies and hepatosplenomegaly. Establishing the diagnosis is done though molecular studies or by fulfilling at least five of the eight signs or symptoms. Prognosis of the disease varies with a high mortality rate if not treated immediately.
Prompt recognition and management of this condition is vital, as a timely initiation of proper chemotherapy can drastically alter the prognosis of patients with this condition.
Keywords: Hemophagocytic, lymphohistiocytosis, infants, immunodeficiency, mortality
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