A Rare case of macrophage activation syndrome as initial presentation of systemic lupus erythematosus treated successfully using 2004 HLH treatment protocol / Kayla Pamela B. de la Cerna.

Contributor(s):

de la Cerna, Kayla Pamela B., MD [author]

Language: english Publication details: Fairview, Quezon City: Department of Internal Medicine, FEU-NRMF, 2020.Description: tables; (in folder) with flash drive (soft copy)Content type:

text

Media type:

unmediated

Carrier type:

volume

LOC classification:

MED 2020 0013

Summary: Abstract: This is a case of a 22 year old male diagnosed with systemic lupus in 2016 whose clinical presentation also fulfilled the criteria for macrophage activation syndrome. Two months prior to the diagnosis of SLE, he presented with thin scaly lesions with erythematous base on the scalp and hairline, followed by fever associated with headache and diplopia, then later developed partial memory loss. He was admitted and workup was done which revealed hepatosplenomegaly, multiple enlarged retroperitoneal and pelvic lymph nodes on CT scan, pancytopenia, hypertriglyceridemia, hyperferritinemia, meningitis with beginning hydrocephalus on cranial CT scan, positive ANA and anti-DsDNA and anticardiolipin. Bone marrow aspirate examination showed scattered histiocytes with hemophagocytic activity. While admitted, he developed epistaxis, disorientation, psychotic behaviors and seizures. Patient satisfied the SLICC criteria for SLE and the criteria for Macrophage Activation Syndrome. Patient was treated with 1 gram methylprednisolone pulse therapy once a day for 3 days, intrathecal methotrexate oral cyclosporine, and hydroxychloroquine. This was followed by using the 2004 treatment protocol for Hemophagocytic Lymphohistiocytosis which included intravenous etoposide, and high dose dexamethasone. The patient was discharged improved after 1 month. HLH treatment protocol was continued and was completed on an outpatient basis. Except for the avascular necrosis of the bilateral hips that developed 2 years after the treatment, no serious infection developed while the patient was on HLH treatment protocol. Throughout the 4 years, 3 episodes of lupus flare (rash, seizures, psychosis) were observed every time tapering of prednisone to less the 10mg once a day was attempted. Treatment for Rituximab was contemplated however, there was no consent given for Rituximab yet. Current maintenance medications of the patient are oral cyclosporin, prednisone 10 mg per day, hydroxychloroquine, aspirin, vitamin D, levetiracetam, olanzapine. No signs of lupus activity was noted during July 2020 check up.

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Holdings
Item type	Current library	Call number	Status	Notes	Date due	Barcode
Room Use	Far Eastern University - Nicanor Reyes Medical Foundation Research	MED 2020 0013 (Browse shelf(Opens below))	Available	with flash drive (soft copy)		R001011

Includes appendices and bibliographical references.

Abstract: This is a case of a 22 year old male diagnosed with systemic lupus in 2016 whose clinical presentation also fulfilled the criteria for macrophage activation syndrome. Two months prior to the diagnosis of SLE, he presented with thin scaly lesions with erythematous base on the scalp and hairline, followed by fever associated with headache and diplopia, then later developed partial memory loss. He was admitted and workup was done which revealed hepatosplenomegaly, multiple enlarged retroperitoneal and pelvic lymph nodes on CT scan, pancytopenia, hypertriglyceridemia, hyperferritinemia, meningitis with beginning hydrocephalus on cranial CT scan, positive ANA and anti-DsDNA and anticardiolipin. Bone marrow aspirate examination showed scattered histiocytes with hemophagocytic activity. While admitted, he developed epistaxis, disorientation, psychotic behaviors and seizures. Patient satisfied the SLICC criteria for SLE and the criteria for Macrophage Activation Syndrome. Patient was treated with 1 gram methylprednisolone pulse therapy once a day for 3 days, intrathecal methotrexate oral cyclosporine, and hydroxychloroquine. This was followed by using the 2004 treatment protocol for Hemophagocytic Lymphohistiocytosis which included intravenous etoposide, and high dose dexamethasone. The patient was discharged improved after 1 month. HLH treatment protocol was continued and was completed on an outpatient basis. Except for the avascular necrosis of the bilateral hips that developed 2 years after the treatment, no serious infection developed while the patient was on HLH treatment protocol. Throughout the 4 years, 3 episodes of lupus flare (rash, seizures, psychosis) were observed every time tapering of prednisone to less the 10mg once a day was attempted. Treatment for Rituximab was contemplated however, there was no consent given for Rituximab yet. Current maintenance medications of the patient are oral cyclosporin, prednisone 10 mg per day, hydroxychloroquine, aspirin, vitamin D, levetiracetam, olanzapine. No signs of lupus activity was noted during July 2020 check up.

Research - Department of Medicine

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