PMID- 26229224
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20150803
LR  - 20181113
IS  - 0300-1652 (Print)
IS  - 2229-774X (Electronic)
IS  - 0300-1652 (Linking)
VI  - 56
IP  - 3
DP  - 2015 May-Jun
TI  - Haematopoietic stem cell transplantation in Nigerian sickle cell anaemia children 
      patients.
PG  - 175-9
LID - 10.4103/0300-1652.160355 [doi]
AB  - BACKGROUND: Sickle cell anaemia (SCA) remains associated with high risks of 
      morbidity and early death. Children with SCA are at high risk for ischaemic 
      stroke and transient ischaemic attacks, secondary to intracranial arteriopathy 
      involving carotid and cerebral arteries. Allogeneic haematopoietic stem cell 
      transplantation (HSCT) is the only curative treatment for SCA. We report our 
      experience with transplantation in a group of patients with the Black African 
      variant of SCA. PATIENTS AND METHODS: This study included 31 consecutive SCA 
      patients who underwent bone marrow transplantation from human leukocyte antigen 
      (HLA)-identical sibling donors between 2010 and 2014 following a 
      myeloablative-conditioning regimen. RESULTS: The median patient age was 10 years 
      (range 2-17 years). Before transplantation, 14 patients had recurrent, painful, 
      vaso-occlusive crisis; ten patients had recurrent painful crisis in association 
      with acute chest syndrome; three patients experienced ischaemic stroke and 
      recurrent vaso-occlusive crisis; two patients experienced ischaemic stroke; one 
      patient exhibited leukocytosis; and one patient exhibited priapism. Of the 31 
      patients, 28 survived without sickle cell disease, with Lansky/Karnofsky scores 
      of 100. All surviving patients remained free of any SCA-related events after 
      transplantation. CONCLUSION: The protocols used for the preparation to the 
      transplant in thalassaemia are very effective also in the other severe 
      haemoglobinopathy as in the sickle cell anaemia with 90% disease free survival. 
      Today, if a SCA patient has a HLA identical family member, the cellular gene 
      therapy through the transplantation of the allogeneic haemopoietic cell should be 
      performed. Tomorrow, hopefully, the autologous genetically corrected stem cell 
      will break down the wall of the immunological incompatibility.
FAU - Isgro, Antonella
AU  - Isgro A
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Paciaroni, Katia
AU  - Paciaroni K
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Gaziev, Javid
AU  - Gaziev J
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Sodani, Pietro
AU  - Sodani P
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Gallucci, Cristiano
AU  - Gallucci C
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Marziali, Marco
AU  - Marziali M
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Angelis, Gioia De
AU  - Angelis GD
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Alfieri, Cecilia
AU  - Alfieri C
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Ribersani, Michela
AU  - Ribersani M
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Roveda, Andrea
AU  - Roveda A
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
FAU - Akinyanju, Olufemi O
AU  - Akinyanju OO
AD  - Sickle Cell Foundation Nigeria, Lagos, Nigeria.
FAU - Wakama, T Thompson
AU  - Wakama TT
AD  - National Hospital, Abuja, Nigeria.
FAU - Olowoselu, Festus Olusola
AU  - Olowoselu FO
AD  - Department of Haematology and Blood Transfusion, College of Medicine, Lagos 
      University Teaching Hospital, Lagos, Nigeria.
FAU - Adediran, Adewumi
AU  - Adediran A
AD  - Department of Haematology and Blood Transfusion, College of Medicine, Lagos 
      University Teaching Hospital, Lagos, Nigeria.
FAU - Lucarelli, Guido
AU  - Lucarelli G
AD  - International Center for Transplantation in Thalassemia and Sickle Cell Anemia, 
      Mediterranean Institute of Hematology, Policlinic Tor Vergata, Rome, Italy.
LA  - eng
PT  - Journal Article
PL  - Nigeria
TA  - Niger Med J
JT  - Nigerian medical journal : journal of the Nigeria Medical Association
JID - 0315137
PMC - PMC4518332
OTO - NOTNLM
OT  - Bone marrow transplantation
OT  - children
OT  - sickle cell anaemia
COIS- Conflict of Interest: None declared.
EDAT- 2015/08/01 06:00
MHDA- 2015/08/01 06:01
PMCR- 2015/05/01
CRDT- 2015/08/01 06:00
PHST- 2015/08/01 06:00 [entrez]
PHST- 2015/08/01 06:00 [pubmed]
PHST- 2015/08/01 06:01 [medline]
PHST- 2015/05/01 00:00 [pmc-release]
AID - NMJ-56-175 [pii]
AID - 10.4103/0300-1652.160355 [doi]
PST - ppublish
SO  - Niger Med J. 2015 May-Jun;56(3):175-9. doi: 10.4103/0300-1652.160355.