000			03050nam a22002537a 4500
999			_c12004 _d12004
001			MED 2020 0013
003			PILC
005			20240720153215.0
008			211022b xxu||||| |||| 00| 0 eng d
040			_beng _cFEU-NRMF MEDICAL LIBRARY _erda
041			_aenglish
050			_aMED 2020 0013
245			_aA Rare case of macrophage activation syndrome as initial presentation of systemic lupus erythematosus treated successfully using 2004 HLH treatment protocol / _cKayla Pamela B. de la Cerna.
260			_aFairview, Quezon City: _bDepartment of Internal Medicine, FEU-NRMF, _c2020.
300			_btables; _c(in folder) _ewith flash drive (soft copy)
336			_2rdacontent _atext
337			_2rdamedia _aunmediated
338			_2rdacarrier _avolume
504			_aIncludes appendices and bibliographical references.
520			_aAbstract: 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.
521			_aRESDM
700			_ade la Cerna, Kayla Pamela B., MD. _eauthor
942			_2lcc _cRU