第四型免疫球蛋白異型症合併腸管結節性淋巴增生過盛之一病例報告

A Case Report of Dysgammaglobulinemia Type Ⅳ Associated with Nodular Lymphoid Hyperplasia of the Intestine

蘇文珍(Wen-Jen Su) ； 謝貴雄(Kue-Hsiung Hsieh) ； 李廷堅(Ting-Chien Lee)

《Acta Paediatrica Sinica》 16卷1期 (1975/03) Pp. 14-17

摘要

病兒係24個月大之男孩，從三個月開始就一直有發燒、腹瀉，一年多來曾得過三次中耳炎而且耳朵流膿。住院身體檢查發現有全身性淋巴結及肝脾腫大，免疫球蛋白檢查顯示IgG 71 mg%，IgA 113 mg%，IgM 430 mg%係屬第四型之免疫球蛋白異型症。大腸X-光及活體組織檢查發現有結節性淋巴增生過盛。兹將其臨床症狀，實驗室檢查及治療經過提出報告，並綜合有關文獻加以論述。

並列摘要

A 24-month-old male baby with recurrent attacks of otitis media, bronchitis, persistent fever and intractable diarrhea found since 3 months of age was noted to have marked hepatosplenomegaly and generalized lymphadenopathy. Serum immunoglobulin check-up showed decreased IgG (71-110 mg%), normal IgA and elevated 1gM. Intestinal nodular lymphoid hyperplasia was proved by both roentgenographic finding of the intestines after barium enema and rectal biopsy. These findings were compatible with the diagnosis of type Ⅳ dysgammaglobulinemia associated with nodular lymphoid hyperplasia. Although antibiotics, steroid, gammaglobulins and plasma transfusion were given during admission, the general condition remained nearly the same. The patient is now being followed up at the allergy and immunologic clinic with monthly administration of plasma and preventive antibiotics.