NK-CELL LYMPHOBLASTIC LEUKAEMIA/LYMPHOMA: CLINICAL AND LABORATORY FEATURES, AND PROGNOSIS
(Abstract release date: 05/19/16)
EHA Library. Antipova A. 06/09/16; 134523; PB1623
Ms. Alina Antipova
Contributions
Contributions
Abstract
Abstract: PB1623
Type: Publication Only
Background
NK-cell lymphoblastic leukemia/lymphoma (NK-LL) is a rare type of acute leukemias. According to the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues NK-LL is reviewed in chapter “Acute leukaemias of ambiguous lineage”.
Aims
To determine clinical and laboratory features and prognosis of NK-LL.
Methods
From 2000 to 2014 only in 1 of 161 (0.6%) patients treated in the department of Hematology of N.N. Blokhin Russian Cancer Research Center was diagnosed NK-LL. Over the same period a diagnosis of NK-LL has been set additionally 3 patients in the Laboratory of Immunology Hematopoietic of N.N. Blokhin Russian Cancer Research Center more. The disease was diagnosed in accordance to the 2008 WHO classification. The differential diagnosis was carried out with other CD56-positive tumors – AML-M0, blastic plasmacytoid dendritic cell neoplasm, T-ALL, and neoplasms of mature NK cells. In the group NK-LL were 4 patients: 3 men and 1 woman. The median age was 52.5 years (ranged from 29 to 82 years).
Results
All patients stated the total bone marrow blast metaplasia (>70%), common extramedullary lesion: generalized lymphadenopathy, hepatosplenomegaly, lesions of skin, tonsils, mediastinal, central nervous system (CNS) by type neuroleukemia. Blasts cytochemical reaction on myeloperoxidase, lipids and nonspecific esterase were negative. In all cases the blast cells were strong positive to CD56 (69.8-99.1%) and the T-associated antigen CD7 (66.2 - 92%), and negative to myeloid, T- and B-lymphoid antigens. PCR identified the lack of gene rearrangement chain T-cell receptor in one patient. Cytogenetic study was not performed. Induction therapy of patients with NK-LL carried out mainly by the program of treatment of acute lymphoblastic leukemia (ALL). CR was achieved in 2 patients, however, proved to be of short-term (1 and 7 months.). The longest remission duration (20 mo.) was obtained using a combined regimen RACOP for the treatment of relapse. Overall survival in the group did not exceed 3 years (1-29 mo.).
Conclusion
The analysis indicates the low efficiency of current regimens NK-LL. Success in treatment of patients depends on prompt and accurate diagnosis, as well as the development of new therapeutic approaches.
Session topic: E-poster
Keyword(s): Acute leukemia, CD56, NK cell
Type: Publication Only
Background
NK-cell lymphoblastic leukemia/lymphoma (NK-LL) is a rare type of acute leukemias. According to the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues NK-LL is reviewed in chapter “Acute leukaemias of ambiguous lineage”.
Aims
To determine clinical and laboratory features and prognosis of NK-LL.
Methods
From 2000 to 2014 only in 1 of 161 (0.6%) patients treated in the department of Hematology of N.N. Blokhin Russian Cancer Research Center was diagnosed NK-LL. Over the same period a diagnosis of NK-LL has been set additionally 3 patients in the Laboratory of Immunology Hematopoietic of N.N. Blokhin Russian Cancer Research Center more. The disease was diagnosed in accordance to the 2008 WHO classification. The differential diagnosis was carried out with other CD56-positive tumors – AML-M0, blastic plasmacytoid dendritic cell neoplasm, T-ALL, and neoplasms of mature NK cells. In the group NK-LL were 4 patients: 3 men and 1 woman. The median age was 52.5 years (ranged from 29 to 82 years).
Results
All patients stated the total bone marrow blast metaplasia (>70%), common extramedullary lesion: generalized lymphadenopathy, hepatosplenomegaly, lesions of skin, tonsils, mediastinal, central nervous system (CNS) by type neuroleukemia. Blasts cytochemical reaction on myeloperoxidase, lipids and nonspecific esterase were negative. In all cases the blast cells were strong positive to CD56 (69.8-99.1%) and the T-associated antigen CD7 (66.2 - 92%), and negative to myeloid, T- and B-lymphoid antigens. PCR identified the lack of gene rearrangement chain T-cell receptor in one patient. Cytogenetic study was not performed. Induction therapy of patients with NK-LL carried out mainly by the program of treatment of acute lymphoblastic leukemia (ALL). CR was achieved in 2 patients, however, proved to be of short-term (1 and 7 months.). The longest remission duration (20 mo.) was obtained using a combined regimen RACOP for the treatment of relapse. Overall survival in the group did not exceed 3 years (1-29 mo.).
Conclusion
The analysis indicates the low efficiency of current regimens NK-LL. Success in treatment of patients depends on prompt and accurate diagnosis, as well as the development of new therapeutic approaches.
Session topic: E-poster
Keyword(s): Acute leukemia, CD56, NK cell
Abstract: PB1623
Type: Publication Only
Background
NK-cell lymphoblastic leukemia/lymphoma (NK-LL) is a rare type of acute leukemias. According to the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues NK-LL is reviewed in chapter “Acute leukaemias of ambiguous lineage”.
Aims
To determine clinical and laboratory features and prognosis of NK-LL.
Methods
From 2000 to 2014 only in 1 of 161 (0.6%) patients treated in the department of Hematology of N.N. Blokhin Russian Cancer Research Center was diagnosed NK-LL. Over the same period a diagnosis of NK-LL has been set additionally 3 patients in the Laboratory of Immunology Hematopoietic of N.N. Blokhin Russian Cancer Research Center more. The disease was diagnosed in accordance to the 2008 WHO classification. The differential diagnosis was carried out with other CD56-positive tumors – AML-M0, blastic plasmacytoid dendritic cell neoplasm, T-ALL, and neoplasms of mature NK cells. In the group NK-LL were 4 patients: 3 men and 1 woman. The median age was 52.5 years (ranged from 29 to 82 years).
Results
All patients stated the total bone marrow blast metaplasia (>70%), common extramedullary lesion: generalized lymphadenopathy, hepatosplenomegaly, lesions of skin, tonsils, mediastinal, central nervous system (CNS) by type neuroleukemia. Blasts cytochemical reaction on myeloperoxidase, lipids and nonspecific esterase were negative. In all cases the blast cells were strong positive to CD56 (69.8-99.1%) and the T-associated antigen CD7 (66.2 - 92%), and negative to myeloid, T- and B-lymphoid antigens. PCR identified the lack of gene rearrangement chain T-cell receptor in one patient. Cytogenetic study was not performed. Induction therapy of patients with NK-LL carried out mainly by the program of treatment of acute lymphoblastic leukemia (ALL). CR was achieved in 2 patients, however, proved to be of short-term (1 and 7 months.). The longest remission duration (20 mo.) was obtained using a combined regimen RACOP for the treatment of relapse. Overall survival in the group did not exceed 3 years (1-29 mo.).
Conclusion
The analysis indicates the low efficiency of current regimens NK-LL. Success in treatment of patients depends on prompt and accurate diagnosis, as well as the development of new therapeutic approaches.
Session topic: E-poster
Keyword(s): Acute leukemia, CD56, NK cell
Type: Publication Only
Background
NK-cell lymphoblastic leukemia/lymphoma (NK-LL) is a rare type of acute leukemias. According to the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues NK-LL is reviewed in chapter “Acute leukaemias of ambiguous lineage”.
Aims
To determine clinical and laboratory features and prognosis of NK-LL.
Methods
From 2000 to 2014 only in 1 of 161 (0.6%) patients treated in the department of Hematology of N.N. Blokhin Russian Cancer Research Center was diagnosed NK-LL. Over the same period a diagnosis of NK-LL has been set additionally 3 patients in the Laboratory of Immunology Hematopoietic of N.N. Blokhin Russian Cancer Research Center more. The disease was diagnosed in accordance to the 2008 WHO classification. The differential diagnosis was carried out with other CD56-positive tumors – AML-M0, blastic plasmacytoid dendritic cell neoplasm, T-ALL, and neoplasms of mature NK cells. In the group NK-LL were 4 patients: 3 men and 1 woman. The median age was 52.5 years (ranged from 29 to 82 years).
Results
All patients stated the total bone marrow blast metaplasia (>70%), common extramedullary lesion: generalized lymphadenopathy, hepatosplenomegaly, lesions of skin, tonsils, mediastinal, central nervous system (CNS) by type neuroleukemia. Blasts cytochemical reaction on myeloperoxidase, lipids and nonspecific esterase were negative. In all cases the blast cells were strong positive to CD56 (69.8-99.1%) and the T-associated antigen CD7 (66.2 - 92%), and negative to myeloid, T- and B-lymphoid antigens. PCR identified the lack of gene rearrangement chain T-cell receptor in one patient. Cytogenetic study was not performed. Induction therapy of patients with NK-LL carried out mainly by the program of treatment of acute lymphoblastic leukemia (ALL). CR was achieved in 2 patients, however, proved to be of short-term (1 and 7 months.). The longest remission duration (20 mo.) was obtained using a combined regimen RACOP for the treatment of relapse. Overall survival in the group did not exceed 3 years (1-29 mo.).
Conclusion
The analysis indicates the low efficiency of current regimens NK-LL. Success in treatment of patients depends on prompt and accurate diagnosis, as well as the development of new therapeutic approaches.
Session topic: E-poster
Keyword(s): Acute leukemia, CD56, NK cell
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