Letterer-siwe disease (LSD) : a case report
Joint Authors
Suad H. H.
al-Amin, Muna Muhammad
al-Sayyid, Khalid Abd al-Muhsin Awad
Madawi, Jad Allah
Source
Sudan Journal of Medical Sciences
Issue
Vol. 13, Issue 3 (30 Sep. 2018), pp.207-218, 12 p.
Publisher
Omdurman Islamic University Faculty of Medicine
Publication Date
2018-09-30
Country of Publication
Sudan
No. of Pages
12
Main Subjects
Pharmacy, Health & Medical Sciences
Abstract EN
Background: Letterer–Siwe Disease (LSD) is one of the variants of Langerhans cell histiocytosis (LCH), which is considered as a rare disease that affects many systems in the body; it is characterized by monoclonal migration and proliferation of specific dendritic cells.
The disease affects the bones and skin primarily, but can involve other organs as well, or appear as a multi-system disease leading to different clinical manifestations and eventually death.
Summary: The authors present a case report of LSD in a two-year-old child from western Sudan, Messeria tribe, who is presented with one and a half-month history of fever, cutaneous ulcers, purprae, scaly crusted scalp, and pallor.
His full blood count showed very low Hb with marked reduction of platelets.
TWBC was normal.
US showed hepatosplenomegaly with lymphadenopathy.
A suspicion of sickle cell anemia and leukemia was suggested.
He received treatment in his area in the form of antibiotics, skin care, blood transfusion and platelets aggregate without improvement.
Patient was referred to Khartoum for further investigations and management.
On presentation, a diagnosis of histiocytosis x was suggested depending on the clinical presentation of a general ill health in a child with purpurae, skin ulcers, and a scaly crusted scalp.
A skin biopsy, bone marrow aspirate, and a skull x-ray were requested.
Bone marrow aspiration showed hyper cellular BM with marked hemophagocytosis.
Patient was admitted in a pediatric ward for further general investigations and blood transfusion, but he passed few days later before starting chemotherapy.
Usually this is the prognosis of this rare and fatal aggressive form of histiocytosis x.
Conclusion: A sick child with fever, anemia, hepatosplenomegaly, scaly scalp, and skin lesions should be investigated for LSD.
American Psychological Association (APA)
Suad H. H.& al-Amin, Muna Muhammad& Madawi, Jad Allah& al-Sayyid, Khalid Abd al-Muhsin Awad. 2018. Letterer-siwe disease (LSD) : a case report. Sudan Journal of Medical Sciences،Vol. 13, no. 3, pp.207-218.
https://search.emarefa.net/detail/BIM-835494
Modern Language Association (MLA)
Suad H. H.…[et al.]. Letterer-siwe disease (LSD) : a case report. Sudan Journal of Medical Sciences Vol. 13, no. 3 (2018), pp.207-218.
https://search.emarefa.net/detail/BIM-835494
American Medical Association (AMA)
Suad H. H.& al-Amin, Muna Muhammad& Madawi, Jad Allah& al-Sayyid, Khalid Abd al-Muhsin Awad. Letterer-siwe disease (LSD) : a case report. Sudan Journal of Medical Sciences. 2018. Vol. 13, no. 3, pp.207-218.
https://search.emarefa.net/detail/BIM-835494
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 217-218
Record ID
BIM-835494