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ASSOCIATION OF MYELOPROLIFERATIVE NEOPLASM AND LYMPHOPROLIFERATIVE DISORDER IN 3 PATIENTS
Author(s):
Barbara FOUCHER
,
Barbara FOUCHER
Affiliations:
Laboratoire,Hopital d'instruction des armées Desgenettes,lyon,France
Marion Dudez
,
Marion Dudez
Affiliations:
Laboratoire,Groupement Hospitalier EST,Lyon,France
Mélanie DANIEL
,
Mélanie DANIEL
Affiliations:
laboratoire,Groupement hospitalier Est,lyon,France
Sandrine GIRARD
,
Sandrine GIRARD
Affiliations:
laboratoire,Groupement Hospitalier Est,LYON,France
Christine FROELICH
,
Christine FROELICH
Affiliations:
laboratoire,Groupement Hospitalier Est,LYON,France
Fanélie MESTRALLET
,
Fanélie MESTRALLET
Affiliations:
laboratoire,Groupement hospitalier Est,LYON,France
Isabelle TIGAUD
,
Isabelle TIGAUD
Affiliations:
laboratoire,Groupement hospitalier Sud,LYON,France
Sandrine HAYETTE
,
Sandrine HAYETTE
Affiliations:
department of cytogenetics and molecular biology,Groupement Hospitalier EST,LYON,France
Amine BELHABRI
,
Amine BELHABRI
Affiliations:
department of oncology and hematology,Centre Leon Berard,LYON,France
Marie Pierre PAGES
,
Marie Pierre PAGES
Affiliations:
laboratoire,Groupement hospitalier Est,LYON,France
Liliana VILA
Liliana VILA
Affiliations:
laboratory,Groupement Hospitalier EST,LYON,France;laboratoire,Centre Léon Bérard,LYON,France
(Abstract release date: 05/18/17) EHA Library. Foucher B. 05/18/17; 182745; PB2031
Barbara Foucher
Barbara Foucher
Contributions
PDF Abstract
Abstract

Abstract: PB2031

Type: Publication Only

Background
Lymphoproliferative disorders (LPD) and myeloproliferative neoplasms (MPN) are two very different sets of hematological pathologies. However, several studies have shown that the risk for LPD onset in patients with MPN is higher than in the general population (1)(2). No single LPD seems to be more at cause and all MPN are likely to present the onset of an associated LPD.

Aims
We present 3 cases diagnosed in the Department of Hematology, « Groupement Hospitalier Est », Lyon, France, of patients bearing an association of MPN and LPD: an essential thrombocythemia (ET) with myeloma, ET with marginal zone lymphoma and a chronic myeloid leukemia with chronic lymphoid leukemia.

Methods
Diagnosis have been made thanks to cytology of peripherical blood, bone marrow aspirate and biopsy and confirmed by cytogenetic and molecular biology techniques.

Results
Case number 1

A 68 year old woman known to have essential thrombocythemia as a MPN, with V617F mutation of the JAK2 protein kinase. After 19 years of treatment by Hydrea, she developed a splenomegaly, anemia and slight lymphocytosis of 4.77 G/L. The blood smear, the bone marrow aspirate and biopsy examination revealed myeofibrosis evolution and an infiltration by 30% of a small sized clonal lymphoid population CD20+, CD5-.Medullar karyotype was normal: 46, XX[10].In conclusion the ET has evolved into myelofibrosis and is associated with a lymphoproliferative syndrome, possibly marginal zone lymphoma.No additional treatment has been implemented.
Case number 2
A 64 year old woman know to have ET with V617F mutation of the JAK2 protein kinase treated by acetic salicylic acid. 5 years after, she presented with IgG kappa type monoclonal gammopathy up to 28 g/L, without any associated clinical manifestation nor cytopenia. Medullar blood was diluted but showed slightly atypical plasmocytes remaining under 10%.Myeloma was diagnosed anyway and the patient received 5 cures of Velcade-Melphalan-Prednisone which resulted in complete remission. The MPN remains stable to this day.
Case number 3
A 62 year old man with chronic lymphoid leukemia, treated by six cycles of R-FC. While in remission since 2 years, hemogram shows hyperleucocytosis (WBC: 18.3 G/L) with thrombocythemia (platelets: 1886 G/L) without anemia (Hb: 13.7 g/dL).Blood smear examination reveals 3% of myelemia and basophilia (3,66 G/L).BCR-ABL transcript is positive in 43% and karyotype points out a 9;22 translocation. (46, XY, t (9 ;22) (q34 ;q11)[1] nuc ish (BLX3, BCRx3,ABL.con BCRX2)[48/100].) Before starting Nilotinib, cytoreductive treatment by Hydrea was decided. Treatment is under way.

Conclusion
The three cases described highlight the diverse situations observed in cases of combined MPN/LPD pathologies. MPN with secondary onset of LPD are most frequently encountered, as was the case with patients 1 and 2. Cases of preexisting LPD and late onset MPN are rare (1), and cases of simultaneous discovery of both pathologies even more so (3).

Several hypotheses have been formulated to explain the frequency of onset of these pathological associations: genomic instability due to JAK2 protein kinase activation, or due to BCR-ABL mutation, or exposure to cytotoxic chemotherapy or radiations (3)

Session topic: 15. Myeloproliferative neoplasms - Biology

Keyword(s): Lymphoproliferative disorder, Myeloproliferative disorder

Abstract: PB2031

Type: Publication Only

Background
Lymphoproliferative disorders (LPD) and myeloproliferative neoplasms (MPN) are two very different sets of hematological pathologies. However, several studies have shown that the risk for LPD onset in patients with MPN is higher than in the general population (1)(2). No single LPD seems to be more at cause and all MPN are likely to present the onset of an associated LPD.

Aims
We present 3 cases diagnosed in the Department of Hematology, « Groupement Hospitalier Est », Lyon, France, of patients bearing an association of MPN and LPD: an essential thrombocythemia (ET) with myeloma, ET with marginal zone lymphoma and a chronic myeloid leukemia with chronic lymphoid leukemia.

Methods
Diagnosis have been made thanks to cytology of peripherical blood, bone marrow aspirate and biopsy and confirmed by cytogenetic and molecular biology techniques.

Results
Case number 1

A 68 year old woman known to have essential thrombocythemia as a MPN, with V617F mutation of the JAK2 protein kinase. After 19 years of treatment by Hydrea, she developed a splenomegaly, anemia and slight lymphocytosis of 4.77 G/L. The blood smear, the bone marrow aspirate and biopsy examination revealed myeofibrosis evolution and an infiltration by 30% of a small sized clonal lymphoid population CD20+, CD5-.Medullar karyotype was normal: 46, XX[10].In conclusion the ET has evolved into myelofibrosis and is associated with a lymphoproliferative syndrome, possibly marginal zone lymphoma.No additional treatment has been implemented.
Case number 2
A 64 year old woman know to have ET with V617F mutation of the JAK2 protein kinase treated by acetic salicylic acid. 5 years after, she presented with IgG kappa type monoclonal gammopathy up to 28 g/L, without any associated clinical manifestation nor cytopenia. Medullar blood was diluted but showed slightly atypical plasmocytes remaining under 10%.Myeloma was diagnosed anyway and the patient received 5 cures of Velcade-Melphalan-Prednisone which resulted in complete remission. The MPN remains stable to this day.
Case number 3
A 62 year old man with chronic lymphoid leukemia, treated by six cycles of R-FC. While in remission since 2 years, hemogram shows hyperleucocytosis (WBC: 18.3 G/L) with thrombocythemia (platelets: 1886 G/L) without anemia (Hb: 13.7 g/dL).Blood smear examination reveals 3% of myelemia and basophilia (3,66 G/L).BCR-ABL transcript is positive in 43% and karyotype points out a 9;22 translocation. (46, XY, t (9 ;22) (q34 ;q11)[1] nuc ish (BLX3, BCRx3,ABL.con BCRX2)[48/100].) Before starting Nilotinib, cytoreductive treatment by Hydrea was decided. Treatment is under way.

Conclusion
The three cases described highlight the diverse situations observed in cases of combined MPN/LPD pathologies. MPN with secondary onset of LPD are most frequently encountered, as was the case with patients 1 and 2. Cases of preexisting LPD and late onset MPN are rare (1), and cases of simultaneous discovery of both pathologies even more so (3).

Several hypotheses have been formulated to explain the frequency of onset of these pathological associations: genomic instability due to JAK2 protein kinase activation, or due to BCR-ABL mutation, or exposure to cytotoxic chemotherapy or radiations (3)

Session topic: 15. Myeloproliferative neoplasms - Biology

Keyword(s): Lymphoproliferative disorder, Myeloproliferative disorder

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