LYMPHOMA ASSOCIATED HEMOPHAGOCYTIC SYNDROME: A SINGLE-CENTER EXPERIENCE.
Author(s): ,
Javier Alberto Rojas Martínez
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Concepción Prats-Martín
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Laura Pérez Ortega
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Rosario Morales Camacho
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Teresa Knight Asorey
Affiliations:
Hematología y Hemoterapia,Hospital Ciudad de Coria,Coria,Spain
,
Olga Pérez- López
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen Macarena,Sevilla,Spain
,
Teresa Caballero-Velázquez
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Fátima De la Cruz-Vicente
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
María Teresa Vargas de los Monteros
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
,
Ricardo Bernal Ruiz
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
Jose Antonio Pérez-Simón
Affiliations:
UGC Hematología y Hemoterapia,Hospital Universitario Virgen del Rocío,Sevilla,Spain
EHA Library. Rojas Martínez J. Jun 15, 2019; 266697; PS1080
Javier Alberto Rojas Martínez
Javier Alberto Rojas Martínez
Contributions
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Abstract

Abstract: PS1080

Type: Poster Presentation

Presentation during EHA24: On Saturday, June 15, 2019 from 17:30 - 19:00

Location: Poster area

Background
Aproximately 1% of the patients with haematological malignancies will develope an hemophagocytic syndrome. Some types of lymphoma are associated with higher risks of developing hemophagocytic syndrome. It is described a high rate of lymphoma associated hemophagocytic syndrome (LAHS) related-mortality. Only few studies have addressed LAHS.

Aims
To describe the clinical and epidemiological features and the prognosis of LAHS in a tertiary hospital from January 2008 to December 2018.

Methods
We performed a ambispective observational study of the cases of hemophagocytic lymphohistiocytosis (HLH) diagnosed by bone marrow aspirate.  The diagnosis was made according to the diagnostic criteria of the  International Histiocyte Society (IHS-2004). Lymphoma classification followed the World Health Organization (WHO) 2017 system. Epidemiological and clinical features of the patients diagnosed of LAHS were analysed.  Statistics performed with SPSS 17.0.

Results
48 patients were diagnosed with HLH in our center in this period. 16 cases (37%) were associated to lymphoma. The median age for patients diagnosed with LAHS was 63 (range: 13-80). The most common histological type was diffuse large B cell lymphoma, which occurred in 7 of 16 patients (44 %). Peripheral T cell lymphoma and Hodgkin lymphoma occurred both in 3 of 16 patients. 7 patients (44%) had other known risk factor for LAHS: viral infection, autoimmune disease and immunosuppression. The clinical and analytical features are summarized in Table 1. The histopathological analysis of the bone marrow did not identify hemophagocytosis in any cases.

Characteristics n % Median  Range

Men

Women

10

 6

63

37

 

Viral infections

 

 

 

HIV

1

6

EBV

3

19

Autoimmmune disease

4

25

 

Kidney transplantation

1

6

 

Splenomegaly

 15

 94

-

-

Fever

14

88

-

-

Cytopenias

16

100

-

-

Hyperferritinemia

14

88

2033 mg/L

362- 40000

Hypertrigliceridemia

12

75

294 mg/L

90-791

Elevated sCD25

4/4

100

35,27  ng/ml

20,70 – 46,37

Hypofibrinogenemia

6

37,5

2,8 mg/dl

0,73- 8,30

Macrophagues (% ) in bone marrow aspirate

1%

0,5-90

 

Hemophagocytosis (%)

25%

0,5-75

 

Most of the patients diagnosed with LAHS (9  of 14) were treated with the first-line chemotherapy regimen for lymphoma. 3 patients were treated following the HLH-2004 protocol. 2 patients died before receiving any treatment.The median overall survival time was 88 days (range: 0 days-6 years). The three months mortality rate was 50%. 4 patients are still alive with 2, 3, 35 and 71 months after the diagnosis of LAHS (Fig 1).

Conclusion
The most common associated lymphoma was diffuse large B cell lymphoma. 44% of the patients had other known risk factor for LAHS. The bone marrow aspirate is superior to the histopathological examination for the diagnosis of LAHS. The three months mortality rate was 50%. Further researches are needed to gain better understanding about this entity.

Session topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical

Keyword(s): Diagnosis, Lymphoma

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