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LYMPHOPENIA IS HIGHLY PREVALENT IN OVERT MYELOFIBROSIS AT DIAGNOSIS AND ASSOCIATED WITH THE DEGREE OF ANEMIA AND THROMBOCYTOPENIA.
Author(s): ,
Nora-Medea Messerich
Affiliations:
Department of General Internal Medicine,Cantonal Hospital St. Gallen,St. Gallen,Switzerland
,
Sergio Cogliatti
Affiliations:
Department of Pathology,Cantonal Hospital St. Gallen,St. Gallen,Switzerland
,
Thomas Lehmann
Affiliations:
Department of Medical Oncology and Hematology,Cantonal Hospital St. Gallen,St. Gallen,Switzerland
,
Andreas Holbro
Affiliations:
Division of Hematology,University Hospital of Basel,Basel,Switzerland
,
Lukas Graf
Affiliations:
Centre for Laboratory Medicine,St. Gallen,Switzerland
Tobias Silzle
Affiliations:
Department of Medical Oncology and Hematology,Cantonal Hospital St. Gallen,St. Gallen,Switzerland
EHA Library. Silzle T. 06/09/21; 324381; PB1708
Tobias Silzle
Tobias Silzle
Contributions
Abstract

Abstract: PB1708

Type: Publication Only

Session title: Myeloproliferative neoplasms - Clinical

Background
Lymphopenia is common in a variety of malignancies, including lymphoma and myelodysplastic syndromes. Little is known about its prevalence in BCR-ABL1-negative myeloproliferative neoplasms (MPN) and its association to prognostic factors or systemic inflammation.

Aims
To evaluate the prevalence of lymphopenia in BCR-ABL-negative MPN at diagnosis and its possible association with disease-related or prognostic factors. 

Methods
Patients diagnosed with a BCR-ABL1-negative MPN between 2010-2020 and having given informed consent were evaluated for the absolute lymphocyte count (ALC) at diagnosis. In addition, parameters of the DIPSS-(plus)-Score and markers of systemic inflammation (albumin, CRP) were retrospectively collected from hospital records. The study was approved by the local ethics committee.

Results
116 patients were included into this preliminary analysis. The median ALC was significantly lower in overt Myelofibrosis (MF) compared to Essential Thrombocythemia (ET), Polycythemia Vera (PV) and prefibrotic PMF (1.3x109/L versus 1.8x109/L, 2.05x109/L and 1.9 x109/L, respectively; p=0.005).

An absolute lymphopenia (defined as ALC <1.0x109/L) was observed in 10/35 patients with overt MF (29%; primary MF n=24, post-ET MF n=3, post-PV MF n=8), 2/35 with ET (6%) and not seen in PV (0/30) and prefibrotic PMF (0/16).


Regarding the single factors of the DIPSS-(plus), the ALC was significantly lower in patients with a hemoglobin < 10 g/l and with platelets < 100x109/L (1.1 vs. 1.6x109/L; p=0.019; and 0.75 vs. 1.5 x109/L; p=0.036, respectively). Patients with leukocytes > 25x109/L (n=3) had higher lymphocyte counts (median 2.5 vs. 1.3 x109/L; p=0.02). For transfusion-dependency, a trend towards a lower ALC was observed (median 0.9 vs. 1.5x109/L, p=0.134). No significant differences of the median ALC were observed for absence/presence of constitutional symptoms, age ≤/> 65 years and peripheral blasts 9/L, intermediate-1 [n=12] 1.4x109/L, intermediate-2 [n=14] 1.2x109/L and high-risk [n=5] 1.9 x109/L; p=0.876). Since metaphase cytogenetics were available in a minority of patients only, the DIPSS-plus could not be evaluated.


Patients with a CRP > ULN (5 mg/l) or an albumin-concentration <35 g/l did not differ in regard to their median ALC (1.5 vs. 1.2x109/L, p=0.32 and 1.4 vs. 1.3x109/L, p=0.886). In addition, there was no difference of the median ALC between the different risk groups of the Glasgow Prognostic Score (GPS), which combines CRP >10mg/l and albumin < 35 g/l (GPS 0 [n=19]: 1.5 x109/L; GPS 1 [n=8]: 1.4 x109/L, GPS 2 [n=3] 1.1x109/L; p=0.927).

Conclusion
Within our MPN cohort, lymphopenia at diagnosis was largely restricted to patients with overt myelofibrosis with considerable prevalence in this entity. Its association with a lower hemoglobin concentration and lower platelet counts points towards a causal relationship of lymphopenia and the pathobiology of the MF-associated bone marrow failure. We were not able to show any influence of systemic inflammation on the ALC or any association of lymphopenia with disease-risk according to DIPSS.  However, interpretation of our data is considerably hampered by the low patient numbers and the retrospective nature of the analysis. Given the rarity of the disease, evaluation of larger (prospective) multicentric patient cohorts would be desirable in order to identify factors related to lymphopenia and to further elucidate its prognostic impact. 

Keyword(s): Inflammation, Lymphocyte, Myelofibrosis, Prognostic factor

Abstract: PB1708

Type: Publication Only

Session title: Myeloproliferative neoplasms - Clinical

Background
Lymphopenia is common in a variety of malignancies, including lymphoma and myelodysplastic syndromes. Little is known about its prevalence in BCR-ABL1-negative myeloproliferative neoplasms (MPN) and its association to prognostic factors or systemic inflammation.

Aims
To evaluate the prevalence of lymphopenia in BCR-ABL-negative MPN at diagnosis and its possible association with disease-related or prognostic factors. 

Methods
Patients diagnosed with a BCR-ABL1-negative MPN between 2010-2020 and having given informed consent were evaluated for the absolute lymphocyte count (ALC) at diagnosis. In addition, parameters of the DIPSS-(plus)-Score and markers of systemic inflammation (albumin, CRP) were retrospectively collected from hospital records. The study was approved by the local ethics committee.

Results
116 patients were included into this preliminary analysis. The median ALC was significantly lower in overt Myelofibrosis (MF) compared to Essential Thrombocythemia (ET), Polycythemia Vera (PV) and prefibrotic PMF (1.3x109/L versus 1.8x109/L, 2.05x109/L and 1.9 x109/L, respectively; p=0.005).

An absolute lymphopenia (defined as ALC <1.0x109/L) was observed in 10/35 patients with overt MF (29%; primary MF n=24, post-ET MF n=3, post-PV MF n=8), 2/35 with ET (6%) and not seen in PV (0/30) and prefibrotic PMF (0/16).


Regarding the single factors of the DIPSS-(plus), the ALC was significantly lower in patients with a hemoglobin < 10 g/l and with platelets < 100x109/L (1.1 vs. 1.6x109/L; p=0.019; and 0.75 vs. 1.5 x109/L; p=0.036, respectively). Patients with leukocytes > 25x109/L (n=3) had higher lymphocyte counts (median 2.5 vs. 1.3 x109/L; p=0.02). For transfusion-dependency, a trend towards a lower ALC was observed (median 0.9 vs. 1.5x109/L, p=0.134). No significant differences of the median ALC were observed for absence/presence of constitutional symptoms, age ≤/> 65 years and peripheral blasts 9/L, intermediate-1 [n=12] 1.4x109/L, intermediate-2 [n=14] 1.2x109/L and high-risk [n=5] 1.9 x109/L; p=0.876). Since metaphase cytogenetics were available in a minority of patients only, the DIPSS-plus could not be evaluated.


Patients with a CRP > ULN (5 mg/l) or an albumin-concentration <35 g/l did not differ in regard to their median ALC (1.5 vs. 1.2x109/L, p=0.32 and 1.4 vs. 1.3x109/L, p=0.886). In addition, there was no difference of the median ALC between the different risk groups of the Glasgow Prognostic Score (GPS), which combines CRP >10mg/l and albumin < 35 g/l (GPS 0 [n=19]: 1.5 x109/L; GPS 1 [n=8]: 1.4 x109/L, GPS 2 [n=3] 1.1x109/L; p=0.927).

Conclusion
Within our MPN cohort, lymphopenia at diagnosis was largely restricted to patients with overt myelofibrosis with considerable prevalence in this entity. Its association with a lower hemoglobin concentration and lower platelet counts points towards a causal relationship of lymphopenia and the pathobiology of the MF-associated bone marrow failure. We were not able to show any influence of systemic inflammation on the ALC or any association of lymphopenia with disease-risk according to DIPSS.  However, interpretation of our data is considerably hampered by the low patient numbers and the retrospective nature of the analysis. Given the rarity of the disease, evaluation of larger (prospective) multicentric patient cohorts would be desirable in order to identify factors related to lymphopenia and to further elucidate its prognostic impact. 

Keyword(s): Inflammation, Lymphocyte, Myelofibrosis, Prognostic factor

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