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العنوان
Clinical and laboratory characteristics of children with pancytopenia attending hematology clinic at alexandria university children’s hospital/
المؤلف
Basha, Mona Mohamed Abd Elaliem.
هيئة الاعداد
باحث / منى محمد عبد العليم باشا
مشرف / أمينه صدقي الحلواني
مشرف / ياسمين فتوح الشاذلي
مشرف / محمد احمد بدر
الموضوع
Pediatrics.
تاريخ النشر
2022.
عدد الصفحات
64 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
الطب
تاريخ الإجازة
10/5/2022
مكان الإجازة
جامعة الاسكندريه - كلية الطب - Pediatrics
الفهرس
Only 14 pages are availabe for public view

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from 78

Abstract

Pancytopenia is a hematological entity in which all blood cell lineages, i.e., leukocytes, erythrocytes, and platelets, are reduced in the blood, leading to anemia, leukopenia, and thrombocytopenia. Pancytopenia is not a disease per se but a manifestation of various diseases affecting the BM or peripheral cell lines. It is diagnosed on the basis of the complete blood count. But, in order to find out the etiological factor causing pancytopenia, a bone marrow examination is usually performed.
This study aimed to report the demographic data, clinical manifestations, etiological factors, final diagnosis, and outcomes of children with pancytopenia attending the hematology clinic at Alexandria University Children Hospital. This retrospective study included 166 children diagnosed with bicytopenia/pancytopenia, representing all the patients meeting the inclusion criteria, who were followed up at the Hematology outpatient clinic until the 31st of December 2020.
The age of the patients at the time of diagnosis ranged from 0.3 to 185.5 months, with a median of 36.4 months. There were 75 (45.2%) males and 91 (54.8%) females with a female to male ratio of 1.2:1. The duration of follow-up since the discovery of pancytopenia was variable ranging from 2.6 to 168.3 months with a median of 41 months.
The most common complaints were pallor (43.1%), fever (16.2%), purpura and ecchymosis (16.2%), gum bleeding (10.8%), abdominal distension (8.5%), failure to thrive (7.7%). The available findings of the patients’ examination revealed that 48 (39.1%) had abnormal anthropometric measurements. Fever was encountered in 24 (15.5%), 59 (37.5%) had purpura, 123 (78.8%) had pallor, 10 (6.3%) had jaundice, 49 (31%) patients had hepatomegaly, 53 (33.7%) had splenomegaly, while only 9 (5.9%) had lymphadenopathy.
A BM aspirate was performed in only 109 patients; 91 (83.5 %) had depressed cellularity, 17 (15.6%) had adequate cellularity, only one patient (0.9%) had increased cellularity. A BM biopsy was performed in 114 patients; 79 (69.3%) had depressed cellularity, 28 (24.6%) had osteopetrosis, three (2.6%) had normal cellularity, two (1.8%) had fibrosis, one (0.9%) had dysplastic BM, another one had a hypercellular BM. The BM cellularity percentage was reported in only 67 patients and ranged from 10% - 70%, with a median of 10%.
Sixty-nine patients (41.6%) were diagnosed with severe aplastic anemia. While 41 (24.7%) as osteopetrosis, 25 (15.1%) as Fanconi anemia, eleven (6.6%) as megaloblastic anemia, four (2.4%) as congenital erythropoietic porphyria, three (1.8%) as congenital megakaryocytic thrombocytopenia, another three (1.8%) were diagnosed as congenital thrombotic thrombocytopenic purpura and one (0.6%) was diagnosed as paroxysmal nocturnal hemoglobinuria.
Owing to different etiologies and clinical pictures of pancytopenia among our patients, 35 patients (21%) received steroids, 27 patients (16.3%) received immunosuppressive therapy, and 26 patients received supplements and multivitamins. Regarding blood transfusions, 27 patients (16.3%) received regular blood transfusions in the clinic, 64 (38.5%) received irregular transfusions, mainly packed RBCs, eight patients received plasma, and 41 received platelets.