Patient With Rare Blood Disorder Successfully Treated At SUM-Bhubaneswar

Bhubaneswar: A 30-year-old woman suffering from a very rare blood disorder was successfully treated at the Institute of Medical Sciences and SUM Hospital recently.

The woman, hailing from Jajpur, was admitted into the hospital in the last week of May for anemia and bleeding requiring repeated blood and platelet transfusions spread over one month. The patient’s bone marrow, the site of blood cell formation, was biopsied for diagnosing the disease and investigations revealed that she was suffering from Severe Aplastic Anemia, a rare blood disorder, where the bone marrow was found completely empty and unable to form new blood cells, Head of Hematology department, Dr Priyanka Samal said.

“Such patients have very low hemoglobin, low total white blood cell count and very low platelet count. They complain of severe weakness, bleeding from orifices or menorrhagia which does not stop unless platelets are transfused,” she said adding these patients also get infected frequently requiring hospitalisation and administration of intra-venous antibiotics and often succumb to sepsis.

Stating that very severe form of Aplastic Anemia had a very poor prognosis and such patients survive only a few months, she further said: “The incidence of this disease is higher in Asia compared to the west and the only cure is available through stem cell transplantation.”

The woman underwent stem cell transplantation on July 5 with stem cells donated by her 28-year-old brother though they had a major blood group discrepancy, which was taken care of very delicately.

Dr Samal said that stem cells in the donor’s body get replaced within 4-6 weeks without any adverse effect on the donor. “A growth factor of 4-5 days is required for the stem cells to get mobilised from the bone marrow to peripheral blood. Following this, apheresis was done for 4-5 hours for harvesting only stem cells from the blood and it was just like a normal platelet donation process,” she said.

The patient was then subjected to high dose chemotherapy after which the donor’s stem cells were infused into her body like normal blood transfusion. It took 12-13 days for these donor cells to make the new blood cells in the patient’s body. The woman, whom the disease might not have given a few months to live, was discharged from the hospital on July 23 in good condition and full blood count recovery though she needs to be closely observed for the next few months for complete immunological recovery, Dr. Samal said.

“The patient can now hope to lead an uneventful life, at least from this ailment’s aspect,” she added.

She also thanked the Medical Superintendent Prof. Pusparaj Samantasinhar, Additional Medical Superintendent Dr Rajesh Lenka, the department of transfusion medicine, laboratory and radiology teams as also the nursing staff whose tireless efforts led to the success of the procedure.