A Rare case of macrophage activation syndrome as initial presentation of systemic lupus erythematosus treated successfully using 2004 HLH treatment protocol / (Record no. 12004)

MARC details
000 -LEADER
fixed length control field 03050nam a22002537a 4500
001 - CONTROL NUMBER
control field MED 2020 0013
003 - CONTROL NUMBER IDENTIFIER
control field PILC
005 - DATE AND TIME OF LATEST TRANSACTION
control field 20240720153215.0
008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION
fixed length control field 211022b xxu||||| |||| 00| 0 eng d
040 ## - CATALOGING SOURCE
Language of cataloging eng
Transcribing agency FEU-NRMF MEDICAL LIBRARY
Description conventions rda
041 ## - LANGUAGE CODE
Language code of text/sound track or separate title english
050 ## - LIBRARY OF CONGRESS CALL NUMBER
Classification number MED 2020 0013
245 ## - TITLE STATEMENT
Title A Rare case of macrophage activation syndrome as initial presentation of systemic lupus erythematosus treated successfully using 2004 HLH treatment protocol /
Statement of responsibility, etc. Kayla Pamela B. de la Cerna.
260 ## - PUBLICATION, DISTRIBUTION, ETC. (IMPRINT)
Place of publication, distribution, etc. Fairview, Quezon City:
Name of publisher, distributor, etc. Department of Internal Medicine, FEU-NRMF,
Date of publication, distribution, etc. 2020.
300 ## - PHYSICAL DESCRIPTION
Other physical details tables;
Dimensions (in folder)
Accompanying material with flash drive (soft copy)
336 ## - CONTENT TYPE
Source rdacontent
Content type term text
337 ## - MEDIA TYPE
Source rdamedia
Media type term unmediated
338 ## - CARRIER TYPE
Source rdacarrier
Carrier type term volume
504 ## - BIBLIOGRAPHY, ETC. NOTE
Bibliography, etc Includes appendices and bibliographical references.
520 ## - SUMMARY, ETC.
Summary, etc. 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.
521 ## - TARGET AUDIENCE NOTE
Target audience note Research - Department of Medicine
700 ## - ADDED ENTRY--PERSONAL NAME
Personal name de la Cerna, Kayla Pamela B., MD.
Relator term author
942 ## - ADDED ENTRY ELEMENTS (KOHA)
Source of classification or shelving scheme Library of Congress Classification
Koha item type Room Use
Holdings
Withdrawn status Lost status Source of classification or shelving scheme Damaged status Not for loan Home library Current library Shelving location Date acquired Total Checkouts Full call number Barcode Date last seen Price effective from Koha item type Public note
    Library of Congress Classification     Far Eastern University - Nicanor Reyes Medical Foundation Far Eastern University - Nicanor Reyes Medical Foundation Research 10/22/2021   MED 2020 0013 R001011 10/22/2021 10/22/2021 Room Use with flash drive (soft copy)