Posterior reversible encephalopathy syndrome (PRES) is one of the most serious complication after allogeneic stem cell transplantation in paediatric setting. It is most commonly reported as adverse event of immunosuppressive strategies during transplantation. We present a case of a 7 years old girl with myelodysplastic syndrome (MDS) treated with allogeneic stem cell transplantation (ASCT) at our department. Diagnosis of PRES was confirmed by imaging techniques during the first month after transplant and it was very likely connected with cyclosporine neurotoxicity. The aim of this article is to present our first experience in diagnosing and treating PRES in paediatric stem cell transplantation. Our experience showed that PRES is one of the reasons for higher transplant related mortality in children. Early prediction of factors contributing to PRES and closely monitoring of patient’s vital signs, especially blood pressure, neurological status and vision are the main contributors for challenging the patient with another immunosuppressive agent that has less neurological toxicity. Still studies have to be initiated to confirm the influence of PRES on transplant outcome.

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BACKGROUND: Allogeneic stem cell transplantation (ASCT) is a potentially curative therapeutic approach in patients with intermediate and high-risk myelodysplastic syndrome (MDS). If a family sibling or unrelated donor is not available mismatched donors are viable option for young patients with no comorbidities. The aim of this case presentation was to evaluate our first experience with haploidentical transplantation for this indication. CASE PRESENTATION: We present a case of 50 years male patient with myelodysplastic syndrome (MDS) diagnosed at University Clinic for hematology, Skopje, North Macedonia. Patient was scored in IPSS -R as high risk patient. He was referred for HLA DNA typing of family siblings and since he didn’t have identical sibling and unrelated donor, he was referred to continue treatment with haploidentical stem cell transplantation. He received Flu Bu conditioning and PTCY, cyclosporine and MMF for GVHD prophylaxis. Peripheral blood stem cells (PBSC) from his mismatched brother were infused in the amount of CD34=5.8x106/kg. He experienced prolonged engraftment, severe infective bacterial infections and CMV reactivation with clinical manifestation of CMV colitis. He was successfully treated with antiviral drug and completely resolved. His bone marrow analysis showed complete remission and chimerism evaluation revealed high donor engraftment. Patient is now +34 months post transplant in complete remission. CONCLUSION: The use of a mismatched donor increases the risk of NRM, but there is also evidence to suggest that an haploidentical donor is a valid choice, as general outcome appears to be at least similar to MUD.

Hairy cell leukemia (HCL) is a rare chronic B-cell malignancy, a slow-growing leukemia that involves the bone marrow, spleen and peripheral blood with hairy cells. The complete blood count may reveal pancytopenia including neutropenia. Splenomegaly is a predominant feature while lymphadenopathy and hepatomegaly are rarely seen. Signs and symptoms of HCL include frequent infections, weakness or feeling tired. The treatment and prognosis of HCL depend on many risk factors such as gender, age, and it is very important to achieve CR which is a critical factor in the success and should be the treatment goal at each stage. Starting from 1984 until 2022 we have observed a total number of 76 patients (61 male, 15 female) with age ranging from 29-83 years, all with HCL. From the total number of patients, 71 (93.4%) were treated with chemotherapy with purine analogs ±, with splenectomy ±, INFα ±, different types of chemotherapy ±, monoclonal antibodies ±. The remaining 5 (6.57%) patients with HCL were only supportive and symptomatically treated. Out of the 71 patients, 50 patients (71.4%) were treated with purine analogs, the second group of 12 patients (16.9%) were treated with different approach without purine analogs, and the third group of 9 (12.7%) were treated with purine analogs and/or splenectomy, INFα, different types of chemotherapy and monoclonal antibodies. The most effective therapy for HCL has proven to be the usage of purine analogs for classical HCL, while for variants of the disease a combination of purine analogs and monoclonal antibodies.

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