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Success Story – Amna Saeed

Age 22 years, Glanzmann Thrombasthenia

Amina Saeed

Contributed By: Mehwish Gillani

Amina Saeed presented with complaints of per rectal bleeding at 06 months of age which was managed conservatively at Jinnah hospital Gujranwala. She again had GI bleeding at 08 months of age and was managed at local Hospital Gujrat. At the age of 5 years, she visited CMH Rawalpindi with complaints of recurrent gum bleed and epistaxis. Platelet Function studies done at AFIP in Feb 2006 was consistent with Glanzmann Thrombasthenia. She received 1 RCC and platelet transfusions and remained transfusions dependent since then, requiring 1-2units RCC every month.

She had an episode of hematemesis in April 2022 and was taken to Ayub lab Rawalpindi where she received 3 injections of NOVO-Seven and was then referred to AFBMTC. She visited AFBMTC on with gum bleed and epistaxis.  She was transfused RCC and was advised HLA typing. Her case was discussed in pretransplant meeting and was enrolled for Allo BMT.

Amina was fully HLA matched with her brother Danish. During her pre transplant work up she was found to be HCV positive. She was started on anti-viral and her PCR for HCV RNA repeated in August 2022 was negative. Amina was admitted at AFBMTC and was transplanted on 29/08/2022 with Bu12.8 (IV) Cy120 used as conditioning therapy. Neutrophil engraftment was achieved on Day +14.

Amina developed sudden onset headache, vomiting followed by generalized tonic clonic fits in Sept 2022 .On arrival at AFBMTC daycare her GCS was 15/15 and her  power was  reduced to 1/5  in left upper and lower limb. She was advised urgent CT brain which showed left thalamic hypo density. Neurology consultation was done. MRI brain with contrast was advised and findings were consistent with atypical posterior reversible encephalopathy syndrome secondary to cyclosporine toxicity. Fits were controlled on day 03 and weakness improved gradually to 5/5 in left upper and lower limb. At discharge, patient was fit free with GCS of 15/15.