الفهرس 
Pathophysiology of Acute lymphoblastic leukemiaOnly 14 pages are availabe for public view
 |  | from | 149 |  |  |
| | | from | 149 | | |
Abstract
A cute lymphoblastic leukemia (ALL) is a cancer of the lymphoid line of blood cells, characterized by the development of large numbers of immature lymphocytes.
The diagnosis of ALL is usually confirmed by A bone marrow aspirate, typically with >20% of all cells being leukemic lymphoblasts.
Patients with acute lymphoblastic leukemia have a poor prognosis, and allogeneic hematopoietic stem cell transplant (AHSCT) is a curative treatment option in these patients.
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) from a matched sibling donor (MSD) is the treatment of choice for high risk patients and relapsed cases.
Currently, TBI _Cy is the most widely used conditioning regimen, since it has acceptable toxicity and is able to kill leukemic cells at protected sites such as the central nervous system (CNS) and testis.
To avoid or diminish the late complications of irradiation, especially in young children, regimens based on chemotherapy alone have been introduced by some researchers The most commonly used chemotherapy regimens for acute leukemia have been the combination of busulfan (BU) and cyclophosphamide (CY).
In this study, we evaluated the outcome of allogeneic PBSCT in 97 patients with(ALL) transplanted from matched sibling donors using a uniform (BU/CY)based conditioning regimen. The patients were recruited from Bone Marrow Transplantation Unit of Nasser Institute Hospital & Ain shams university Hospitals in the time period from December 1997 to May 2015.
All included patients had evidence of BM CR
. The mean age at transplant was 20.3 years in males 18 y in females (± 9.6 SD); ranged from 2years to 48 years.
The conditioning consisted of Busulfan, Cyclophosphamide (CY) was given at a dose of 30 mg / kg / day.
Busulfan(BU):-dose 16 mg /Kg total dose D-11 to D-8(in patients older than 8 y) &20 mg/kg total dose (in patients less than 8y).
For graft-versus-host disease (GVHD) prophylaxis we used cyclosporine-A at a starting dose of 3mg/kg then tailored according to trough level.MTXwas administered in for additional GVHD prophylaxis MTX was given at a dose of 15mg/kg/day, on(D +1), 10mg /KG on D +3, D +6 and D +11.
The mean CD 34+ included was 5 x 106/kg recipient weight.
Acute GVHD (aGVHD) was observed in (20%) of cases (n=19).(47%) of them developed aGVHD skin only (grade 1) and responded to treatment with topical steroids(n=9)
(9%) developed a GVHD skin and GIT(grade 2-3) (n=9).
(5%) had aGVHD skin.GIT and liver(grade 4) (n=1)
But all responded to treatment with steroid and cyclosporine A.
Chronic GVHD occurred in (9.2%) (n=9), extensive cGVHD (6.1%) (n=6)limited cGVHD (2.9%) (n=3), all of them are 2ry to a GVHD grade II and no primary (de novo) cases were detected
CMV reactivation occurred only in 10% patients (n=10) with normal CMV status in 87 patients.
All patients with CMV reactivation responded well to Gancyclovir treatment and achieved negative CMV status at the end of the course.Over the study follow up period the relapse rate was found to be only 16% (n=16). complete remission rate was 81% (n=81)
Overall Survival (OS) at end of the study period was 52%.
We conclude that the combination of BU, CY in the doses used in this study was agood alternative to TBI-Based regiemns especially for young adults and pediatric patients with good outcome and lower toxicity in of ALL patients undergoing Allo- BMT. The regimen was well tolerated and was associated with a low incidence of complications including GVHD.