Histiocytosis disorders in Northeast Egypt: epidemiology and survival studies (a 5-year study).
Histiocytosis disorders include a wide group of disorders characterized by monocytes, macrophages and dendritic cell infiltration of different tissues. There are few clinico-epidemiologic studies of such disease. Our study was designed to look at the clinico-epidemiological features and outcome of patients with histiocytosis disorders in Northeast Egypt. Twenty-seven cases with histiocytosis disorders accrued over a 5-year period were analyzed and classified as having unifocal, multifocal, or multisystem disease. They were 14 males and 13 females. Twenty-two patients representing 81.5% of cases were more than two years of age while 5 patients (18.5%) were less than 2 years. Lymphadenopathy was the commonest presentation (55.55%) followed by bone lesions (44.44%). Involvement was unifocal in 12, multifocal in 10, and multisystem in 5 cases. The histological features were relatively uniform regardless of the clinical severity, and consisted of Langerhans cells, eosinophils, histiocytes, plasma cells, giant cells and fibrosis. The treatment consisted of a combination of surgery, chemotherapy, and/or radiotherapy. Lymphadenopathy was the most common clinical presentation in our locality. Response to treatment was poor in patients with multisystem disease. Patients with age less than 2 years were more likely to have increased risk of morbidity and mortality, due to widespread disease.
Al-Tonbary
Youssef A
YA
Mansoura University Children's Hospital, Faculty of Medicine, Mansoura University, Mansoura, Egypt. ytonbary@gmail.com
Sarhan
Mohamed M
MM
Mansour
Ahmed K
AK
Abdelrazik
Nabil M
NM
El-Ashry
Rasha A
RA
D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004534:Egypt / Q000453:epidemiology; D005260:Female; D015726:Giant Cells / Q000473:pathology / Q000502:physiology; D015614:Histiocytosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007801:Langerhans Cells / Q000473:pathology; D007962:Leukocytes / Q000473:pathology; D008137:Longitudinal Studies; D008297:Male; D015996:Survival Rate