Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report.
Yumna NjoumLila H Abu-HilalFarah JabbarinSami BannouraSameer MtourMuaath ItmaizehPublished in: Oxford medical case reports (2023)
Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatosplenomegaly and rash. After thorough investigation, RDD was diagnosed by mediastinal lymph node biopsy that revealed presence of S100 and CD68-positive cells with absence of CD1a confirming the diagnosis of RDD. Treatment involved combination of steroids and Rituximab, which proved to be highly effective. The patient had dramatic improvement and entered remission, with follow-up period of 2 years. It is important to note that although RDD is a rare disease, it causes severe complications, as evidenced by the patient's parameters. Thus, prompt diagnosis and treatment are paramount. Histological diagnosis is of great value, as it helps confirming and guiding treatment decisions. With the right treatment, patients can experience great recovery and quality of life.
Keyphrases
- lymph node
- chronic kidney disease
- end stage renal disease
- case report
- squamous cell carcinoma
- single cell
- signaling pathway
- diffuse large b cell lymphoma
- mental health
- mesenchymal stem cells
- induced apoptosis
- early onset
- cell proliferation
- peritoneal dialysis
- cell cycle arrest
- endoplasmic reticulum stress
- fine needle aspiration
- ulcerative colitis
- smoking cessation
- pi k akt