Acute polymyositis associated with hemophagocytic lymphohistiocytosis syndrome

Karjigi, Uma; Plant, Michael

doi:10.1186/1471-2474-14-s1-a8

Cited by 2 publications

(3 citation statements)

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“…3,4 Rheumatological diseases have been linked to a significant number of cases of HLH around the world, with systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA) being the most common; but very few cases have been reported with polymyositis. 5 Our case report aims to highlight a rare and unique association between HLH and polymyositis so that the disease is identified and treated early and effectively to assure long-term survival.…”

Section: Introductionmentioning

confidence: 99%

Case Report: Hemophagocytic lymphohistiocytosis associated with acute polymyositis

Iktidar¹,

Jabin

Khan

et al. 2022

F1000Res

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Hemophagocytic lymphohistiocytosis (HLH) is a rare condition marked by uncontrolled histiocyte proliferation and activation, and phagocytosis of normal hematopoietic cells. This disease is rare, and a concurrent presentation with acute polymyositis is rare. A 14-year-old male was admitted to Chattogram Medical College Hospital with a high-grade fever for 25 days, generalized severe body aches, and multiple large joint pain in the lower limb for the same duration. On examination, the patient was found to have splenomegaly, tenderness in both knee joints, symmetrical proximal weakness of both lower limbs, several sensory losses, and loss of bowel and bladder control. The laboratory data showed that he had anemia, thrombocytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, hypoalbuminemia, raised D-dimer, and serum creatine phosphokinase (S. CPK). Along with these, a bone marrow biopsy revealed hemophagocytic cells, and he was diagnosed with HLH with acute polymyositis (PM). The patient was treated with intravenous (IV) hydrocortisone and dexamethasone. Clinical stability was achieved with gradual improvement of initial symptoms and biochemical markers. The patient was discharged with oral steroids at a tapering dose and was advised to regularly follow-up.

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Section: Introductionmentioning

confidence: 99%

Case Report: Hemophagocytic lymphohistiocytosis associated with acute polymyositis

Iktidar¹,

Jabin

Khan

et al. 2022

F1000Res

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show abstract

“…Hemophagocytic lymphohistiocytosis (HLH) is characterized by excessive immune activation. 1 It involves the activation of lymphocytes and histiocytes that infiltrate various organs and release cytokines. 1 A 60-year-old man presented with left hip swelling, heat sensation, tenderness, and fever.…”

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confidence: 99%

“…1 It involves the activation of lymphocytes and histiocytes that infiltrate various organs and release cytokines. 1 A 60-year-old man presented with left hip swelling, heat sensation, tenderness, and fever. He had a history of recurrent fever, anemia, arthralgia, and inflammation at various sites, but no bacteria were found in tissue or blood samples.…”

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confidence: 99%