Irfan Omar, Mahmood Sana, Nand Heera, Billoo Gaffar
Medical College, Aga Khan University Hospital, Karachi, Pakistan.
Department of Pediatrics, Aga Khan University Hospital, Karachi, Pakistan.
J Med Case Rep. 2018 Jan 23;12(1):16. doi: 10.1186/s13256-017-1527-5.
Celiac disease may present with hematological abnormalities including long-standing anemia. Both aplastic anemia and celiac disease have a similar underlying autoimmune process but an association between the two is seldom reported. There have only been three pediatric cases reporting this association and this case is the first reported in a female pediatric patient.
We report a case of 6-year-old South Asian girl presenting with bruises, petechiae, and recent history of loose stools. On evaluation, she was diagnosed as having celiac disease and was put on a gluten-free diet and further investigations including bone marrow biopsy revealed pancytopenia. She was managed with packed red cells, platelets, and diet restrictions and had improving platelet counts over yearly follow ups. Her parents were counseled regarding the need for bone marrow transplant.
This is the fourth case report suggesting an association between celiac disease and aplastic anemia in the pediatric population and this association could be more common than expected. Timely intervention of either celiac disease through strict gluten-free diet or aplastic anemia through immunosuppressive therapy could potentially reduce the risk for other autoimmune conditions. We can see that all four pediatric cases reported with this potential association are from South East Asia and hence larger studies would be prudent to explore this association.
乳糜泻可能伴有血液学异常,包括长期贫血。再生障碍性贫血和乳糜泻都有相似的潜在自身免疫过程,但两者之间的关联鲜有报道。仅有三例儿科病例报告了这种关联,而本例是首例报道的儿科女性患者。
我们报告一例6岁南亚女孩,有瘀斑、瘀点,近期有腹泻病史。经评估,她被诊断为乳糜泻,并开始接受无麸质饮食,进一步检查包括骨髓活检显示全血细胞减少。她接受了浓缩红细胞、血小板治疗及饮食限制,在每年的随访中血小板计数有所改善。已向其父母咨询骨髓移植的必要性。
这是第四例提示儿科人群中乳糜泻与再生障碍性贫血存在关联的病例报告,这种关联可能比预期更为常见。通过严格的无麸质饮食对乳糜泻进行及时干预,或通过免疫抑制疗法对再生障碍性贫血进行及时干预,可能会降低发生其他自身免疫性疾病的风险。我们发现,所有四例报告有这种潜在关联的儿科病例均来自东南亚,因此进行更大规模的研究以探究这种关联是明智的。