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A FAMILIAL REPORT OF MIXED CELLULARITY HODGKIN’S DISEASE(HD): THE SAME SUBTYPE HD, EBV NEGATIVE, WITH DIFFERENT OUTCOME.
Author(s): ,
Maria Moschovi
Affiliations:
Hematology-Oncology Unit, 1st Department of Pediatrics,National and Kapodistrian University of Athens,Athens,Greece
,
Alexandros Lagos
Affiliations:
Hematology-Oncology Unit, 1st Department of Pediatrics,National and Kapodistrian University of Athens,Athens,Greece
,
Charoula Tsipou
Affiliations:
Hematology-Oncology Unit, 1st Department of Pediatrics,National and Kapodistrian University of Athens,Athens,Greece
Natalia Tourkantoni
Affiliations:
Hematology-Oncology Unit, 1st Department of Pediatrics,National and Kapodistrian University of Athens,Athens,Greece
(Abstract release date: 05/17/18) EHA Library. Moschovi M. 06/14/18; 216575; PB2024
Maria Moschovi
Maria Moschovi
Contributions
Abstract

Abstract: PB2024

Type: Publication Only

Background

Mixed cellularity Hodgkin’s disease(HD) is a subtype of classic HD constituting the second most common type and 15-30% of all cases of HD. It is mostly observed in patients with either Epstein-Barr virus (EBV) or Human Immunodeficiency Virus (HIV) infection, having less inheritance rate comparing to other subtypes.

Aims
Presentation of two identical cases among siblings with different outcome.

Methods
Two siblings, a girl and a boy, were presented with mixed cellularity Hodgkin disease. Both, had the same histological subtype, the same stage(IIIs), the same location of the disease and both, at the biopsy specimen, where negative for EBV infection. The only apparent difference between the two cases was the presenting age. The girl was diagnosed at the age of 15 years old and the boy at the age of 25 years old. Both of them received chemotherapy. The girl achieved remission with chemotherapy only. The boy received initially only chemotherapy. Due to the relapsed disease during chemotherapy, radiotherapy was added but he succumbed from refractory disease.

Results
The two cases were siblings. They had the same histological subtype of the disease, the same location, different age of onset but eventually different outcome. The girl received chemotherapy according to the SIOP HD protocol, and till now she is in first remission, 10 years after the diagnosis. By contrast, the boy had a poor outcome.

Conclusion

Considering that both cases where identical on their anatomical and histological characteristics and also both of them where negative for EBV infection, we conclude that the different age of onset of this subtype of HD, and the different gender, play a crucial role on the outcome. Local genetic predisposing factors may contributed to these familial HD cases.

Session topic: 17. Hodgkin lymphoma – Clinical

Keyword(s): Childhood, familial, Hodgkin's Lymphoma

Abstract: PB2024

Type: Publication Only

Background

Mixed cellularity Hodgkin’s disease(HD) is a subtype of classic HD constituting the second most common type and 15-30% of all cases of HD. It is mostly observed in patients with either Epstein-Barr virus (EBV) or Human Immunodeficiency Virus (HIV) infection, having less inheritance rate comparing to other subtypes.

Aims
Presentation of two identical cases among siblings with different outcome.

Methods
Two siblings, a girl and a boy, were presented with mixed cellularity Hodgkin disease. Both, had the same histological subtype, the same stage(IIIs), the same location of the disease and both, at the biopsy specimen, where negative for EBV infection. The only apparent difference between the two cases was the presenting age. The girl was diagnosed at the age of 15 years old and the boy at the age of 25 years old. Both of them received chemotherapy. The girl achieved remission with chemotherapy only. The boy received initially only chemotherapy. Due to the relapsed disease during chemotherapy, radiotherapy was added but he succumbed from refractory disease.

Results
The two cases were siblings. They had the same histological subtype of the disease, the same location, different age of onset but eventually different outcome. The girl received chemotherapy according to the SIOP HD protocol, and till now she is in first remission, 10 years after the diagnosis. By contrast, the boy had a poor outcome.

Conclusion

Considering that both cases where identical on their anatomical and histological characteristics and also both of them where negative for EBV infection, we conclude that the different age of onset of this subtype of HD, and the different gender, play a crucial role on the outcome. Local genetic predisposing factors may contributed to these familial HD cases.

Session topic: 17. Hodgkin lymphoma – Clinical

Keyword(s): Childhood, familial, Hodgkin's Lymphoma

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