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SEVERE HAEMOPHILIA A DIAGNOSED IN A CHILD WITH ALL: FIRST REPORT IN GREECE
Author(s): ,
Maria Ioannidou
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Maria Palabougiouki
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Eleni Thomaidou
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Athanasios Bangeas
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Athanasios Tragiannidis
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Theodotis Papageorgiou
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Marina Economou
Affiliations:
Bleeding Disorders Centre, 1st Department of Pediatrics,School of Medicine, Faculty of Health Sciences, Hippokration General Hospital,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Aikaterini Teli
Affiliations:
Bleeding Disorders Centre, 1st Department of Pediatrics,School of Medicine, Faculty of Health Sciences, Hippokration General Hospital,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Vasiliki Tsavdaridou
Affiliations:
Department of Immunology, Microbiology Laboratory, AHEPA University Hospital,Aristotle University of Thessaloniki,Thessaloniki,Greece
,
Assimina Galli-Tsinopoulou
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
Emmanuel Hatzipantelis
Affiliations:
Children & Adolescent Haematology-Oncology Unit, 2nd Department of Pediatrics, School of Medicine, Faculty of Health Sciences, AHEPA University General Hospital of Thessaloniki,Aristotle University of Thessaloniki,Thessaloniki,Greece
EHA Library. Ioannidou M. 06/09/21; 324061; PB1376
Maria Ioannidou
Maria Ioannidou
Contributions
Abstract

Abstract: PB1376

Type: Publication Only

Session title: Acute lymphoblastic leukemia - Clinical

Background
The coincidence of acute lymphoblastic leukaemia (ALL) and hemophilia is extremely rare in pediatric age group.

Aims
We report the 1st case in Greece with severe haemophilia A  diagnosed in a child with ALL.

Methods
A 2,5 year old boy admitted to our Unit due to generalized weakness and episodes of severe nose bleeding for the last month. Family history revealed that boy’s uncle (mother’s brother) suffered from haemophilia A. From physical examination, paleness, petechiaes and ecchymoses were noticed, as well as, organomegaly. Peripheral blood count showed hemoglobin level of 5.6 g%, white cell count of 212 × 109/L (blasts 95%), and platelet count of 16 × 109/L. Activated partial thromboplastin time (APTT) found very high, but prothrombin time (PT) not.

Results
Bone marrow aspiration-immunophenotype confirmed the diagnosis of B-II (common) ALL. RT-PCR for genetic alterations BCR abl, AF-4/MLL and TEL-AML1 found negative. CSF was also negative for blastic cells. Following the placement of central venous catheter prolonged bleeding was noticed. Prolonged APTT and family history led to the possibility of hemophilia which finally diagnosed from laboratory tests (FVIII levels <1%). The patient was placed on induction therapy for ALL, according to ALLIC 2009 protocol, and FVIII replacement. On day 33 of treatment, the patient achieved complete remission. He finished successfully the first 6 months of treatment and he will hopefully continue on maintenance phase of the protocol.

Conclusion
The most common haemorrhagic disorder in children with leukaemia is disseminated intravascular coagulation (DIC). The diagnosis of haemophilia A is very rare and there is no clear therapeutic plan in such cases, but timely diagnosis should not be delayed and proper treatment should be based on clinical findings.

Keyword(s): Acute lymphoblastic leukemia, Hemophilia A, Pediatric

Abstract: PB1376

Type: Publication Only

Session title: Acute lymphoblastic leukemia - Clinical

Background
The coincidence of acute lymphoblastic leukaemia (ALL) and hemophilia is extremely rare in pediatric age group.

Aims
We report the 1st case in Greece with severe haemophilia A  diagnosed in a child with ALL.

Methods
A 2,5 year old boy admitted to our Unit due to generalized weakness and episodes of severe nose bleeding for the last month. Family history revealed that boy’s uncle (mother’s brother) suffered from haemophilia A. From physical examination, paleness, petechiaes and ecchymoses were noticed, as well as, organomegaly. Peripheral blood count showed hemoglobin level of 5.6 g%, white cell count of 212 × 109/L (blasts 95%), and platelet count of 16 × 109/L. Activated partial thromboplastin time (APTT) found very high, but prothrombin time (PT) not.

Results
Bone marrow aspiration-immunophenotype confirmed the diagnosis of B-II (common) ALL. RT-PCR for genetic alterations BCR abl, AF-4/MLL and TEL-AML1 found negative. CSF was also negative for blastic cells. Following the placement of central venous catheter prolonged bleeding was noticed. Prolonged APTT and family history led to the possibility of hemophilia which finally diagnosed from laboratory tests (FVIII levels <1%). The patient was placed on induction therapy for ALL, according to ALLIC 2009 protocol, and FVIII replacement. On day 33 of treatment, the patient achieved complete remission. He finished successfully the first 6 months of treatment and he will hopefully continue on maintenance phase of the protocol.

Conclusion
The most common haemorrhagic disorder in children with leukaemia is disseminated intravascular coagulation (DIC). The diagnosis of haemophilia A is very rare and there is no clear therapeutic plan in such cases, but timely diagnosis should not be delayed and proper treatment should be based on clinical findings.

Keyword(s): Acute lymphoblastic leukemia, Hemophilia A, Pediatric

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