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Secondary myelodysplastic syndrome in lymphoproliferative diseases
20 ELECTRON A STUDY
Second International
Conference on Myelodysplastic Syndromes
MICROSCOPIC OBSERVATIONS ON BONE MARRI3w: OF 60 MYELODYSPLASTIC SYNDROMES (MDS).
D. Irrlgulble, T. Vallespl. ht. Torrabadella, M.J. de las Heras. J&n, Hoepltal Department of Haematology. d’Hebr6n”. 08036 Barcelona, Spaln.
A. “Vail
syndrome The dlagnorls of myelodysplastlc (MDS) 1s malnly based on rhe flndlng of dysmyelopolerlc features. We srudled 60 MDS parlents with electron transmlsslon microscopy. FAB dlstrlbutlon was: 6 refractory anaemla (RA), 6 RA wlth ring 24 RA with excess of blasts slderoblasts (RAS), (RAEB). 6 RAEB In translormatlon (RAEB-t) and 9 chronii myelomonocytlc leukaemla (CMHL). DYSERYTHROPOIESIS was found In most patients. The mltochondrla were abnormally large and Irregularly wlth Iron deposits dlsorganlzlng the shaped, These phenomena were found mltochondrla crests. In some RAEB. RAEB-t. and CMML, and In all RAS, observed Other abnormalltles none In RA cases. Increased number of rhopheocytosis In MDS were: vesicle and slderosomes, isolated rlbosome, llpldlc detachment of chromatin, vacuola, spongy and enlargement of nuclear perlnucleor cletern, pores. DYSGRANULOPOIESIS was observed In 80% of MDS. Pseudo-Pelger shapes, parallel dlsposltlon of of nuclear pockets, vacuoles, Increase RER, clumping chromatln, dllared Colgl hypogranulatlon, and alterarlon of primary granulation In blasts cells Type II were the most common flndlngs. DYSMECAKARYOCYTOPOIESIS was seen In all subtypes. Frequent presence of mlcromegakaryocyres mononucleated or blnucleared, alreratlon of dlstrlburlon of demarcation membranes and dllatatlons of the were of the platelets canallcular system open observed.
MYELODYSPLASTIC SYNDROME ASSOCIATED WITH CHRONIC LYMPHOCYTICLEUKAEYIA: A REPORT OF THREE CASES. C. Rulz-Marcellhn’, D. T. Vallespl, M. Torrabadella, Irrlguible. A. JaBn, and M. Saris’. Departments of Haematology and (‘1 Pathology. Hospital “Vail d’Hebr6n”. 08036 Barcelona, Spaln. Most frequently described assoclarlons between lymphoid myelodyaplaat.Ic syndromes (MDS) and mallgnanclea Include plasma cell tumours and B- and T-cell lymphomae. We have observed three cases of MDS: 1 refractory anaemla (RA). 1 RA wlth ring alderoblasta (RAS), and 1 RA wlth excess of blasts with (RAEB) coexletlng chronic lymphocytlc leukaemla (CLL) of B lineage (CDlQ+, CD6+, SmIg+ cells). In two cases both dlseases were diagnosed simultaneously. In one case, the patlent was prevlously dlagnosed of RA (Hb 66 g/L, WBC 4.1 x 100 /L, platelets 315x10e/L wlth an hypercellular bone marrow showlng dyserythropolesls, marked dysgranulopoiesis and dysmegakaryopolesls). In the study of bone marrow histology, besldes ofher MDS features, some lymphold aggregates were observed. In splte of sterold Twenty-slx months later, this patlent presented with a perlpheral therapy, blood picture typlcal of CLL (WBC 21.3xlOe/L. 42% Lymphocytes were CD19+ (7490 and lymphold cells). showed SmIg (96% weak posltlvlty). Retrospective etudles uslng monoclonal antlbodles (LN2, MB2 from Clonab. and anti-HLA-DR) agalnst antigens preserved In parafln-embedded samples proved the B nature of at the tlme of the RA lymphold aggregates, dlagnosls. These observations support that, In some cases, CLL may arise from plurlpotentlal stem cells.
SECONDARY
MYELODYSPLASTIC
LYMPHOPROLIFERATIVE
SYNDROME IN
DISEASES.
K.Tm&n$, P.Cieslar’, I SpiEka’, R.Neuwirtod’ and Cze hoslovak WS Cooperative Group; 4st Hed Dept, Dept HaePatol, s Dept Clin Hsematol, 1st Medical Faculty, Charles University, u neaaocnice 2, CS-12808 Prague, Czechoslovakia Several studies have shorn that aponoclonsl gammopathiesare nore frequent in myelodysplastic syndrome in comparison with controls. 36 cases of secondary HDSfolloved by CzechoslovakHDS
CooperativeGroup were studied. 14 cases (3S,9r) were preceeded by lymphoproliferative disease and in 11 (30,6l) cases out of themwas foti monoclonalgarmopathy. This group consists of 9 cases of plasrocytoaa (25,Ot) and 2 cases of non-liodgkin’s lymphoma- impunocytoma with monoklonal gallpopathy (5,6%). Diagnosis wasestablished in 10 cases as RA,in 2 as RASand in 2 as RUB. All patients were treated by chemotherapyand someby actinotherapy as well. Bowevertwo cases whereBIDS developedvery fastly during 3 months(after one cycle of Alkeran therapy) were observed. Transformationof HDSto acute leuktia was observed in 4 cases. The mean interval from diagnosis of monoclonal gammopathy to HDSwas 49 months. Solwinteresting data were found in this group in comparison to the remaining 22 patients with secondary NB9with other origin. 10 cases (66,78) of all RA patients and only 2 patients (14,39) of all RUBpatients were observed in this group. Hediansurvival after HDSdiagnosis was longer in this group with lymphoproliferative diseases (8 manths v. 6,5). Influence of immunological abnormalities on onset of secondaryHDSis discussed.
ANALYSIS OF 36 CASES OF MYELODYSPLASTIC SYNDROME
SECONDARY
K.Trn&I)il,P.Ciesla+, I.Qpifika’, RJeuwirtovi2 and Cz hoslovak “i Dspt Clin HDS- CooperativeGroup. 1st DeptHed, Dept Haematol, Haematol,1st Hedical Faculty, Charles University, U nelaocnice2, Cs-128OSPrague, Czechoslovakia CzechoslovakHDS- cooperative group followed 627 patients with HDStill 1990. Diagnosisof secondaryHDSwas established in 36 (5,7%) patients. RAwasobserved in 15 cases (41,7t), RASin 4 cases (ll,l!), RAF8in 14 (38,9#), RUB-t in 2 (5,6%)and CM in 1 (2,SO). There were 25 females and 11 males in the group, age medianwas 64 years. Professional exposure to X-ray irradiation was observed in 2 cases and to chemicals in 1 case in their previous history. Haematological neoplasia vas observed in 15 cases (14 cases of lymphoproliferative disease, 11 with rtonoclonalgamaopatby), aplastic anaemiain 2 cases, cancer in 13 cases, autoimmune disease in 2 cases, inflamtory disease in 1 case. The occurenceboth carcinoaa and secondaryWS (RAand RUB) after exposure to myelotoxic drugs and chenicals was observed in two cases. In previous history of these patients 13 were treated by myelosuppressivedrugs, 9 by actinotherapy, 7 by both measures, 4 by immunosuppressive therapy. Howeververy fast development of secondary WS after minimaldoses of cytotoxic drugs were observed in 3 cases. Pathological cytcgenetic findings were found in 90,5t cases, at least one typical abnormality (5VI-517(1-, -7) in 73,7#. Hedian interval betweenaetiological factor and onset of HDSwas 48 months, mediansurvival of patients with developedWS was 9 months.Transformationto acute leukaeaia wasobserved in 11 (30,6i) cases. Comparisonto data of the wholegroup of HDSis made.
Second International
Conference on Myelodysplastic Syndromes
MICROSCOPIC OBSERVATIONS ON BONE MARRI3w: OF 60 MYELODYSPLASTIC SYNDROMES (MDS).
D. Irrlgulble, T. Vallespl. ht. Torrabadella, M.J. de las Heras. J&n, Hoepltal Department of Haematology. d’Hebr6n”. 08036 Barcelona, Spaln.
A. “Vail
syndrome The dlagnorls of myelodysplastlc (MDS) 1s malnly based on rhe flndlng of dysmyelopolerlc features. We srudled 60 MDS parlents with electron transmlsslon microscopy. FAB dlstrlbutlon was: 6 refractory anaemla (RA), 6 RA wlth ring 24 RA with excess of blasts slderoblasts (RAS), (RAEB). 6 RAEB In translormatlon (RAEB-t) and 9 chronii myelomonocytlc leukaemla (CMHL). DYSERYTHROPOIESIS was found In most patients. The mltochondrla were abnormally large and Irregularly wlth Iron deposits dlsorganlzlng the shaped, These phenomena were found mltochondrla crests. In some RAEB. RAEB-t. and CMML, and In all RAS, observed Other abnormalltles none In RA cases. Increased number of rhopheocytosis In MDS were: vesicle and slderosomes, isolated rlbosome, llpldlc detachment of chromatin, vacuola, spongy and enlargement of nuclear perlnucleor cletern, pores. DYSGRANULOPOIESIS was observed In 80% of MDS. Pseudo-Pelger shapes, parallel dlsposltlon of of nuclear pockets, vacuoles, Increase RER, clumping chromatln, dllared Colgl hypogranulatlon, and alterarlon of primary granulation In blasts cells Type II were the most common flndlngs. DYSMECAKARYOCYTOPOIESIS was seen In all subtypes. Frequent presence of mlcromegakaryocyres mononucleated or blnucleared, alreratlon of dlstrlburlon of demarcation membranes and dllatatlons of the were of the platelets canallcular system open observed.
MYELODYSPLASTIC SYNDROME ASSOCIATED WITH CHRONIC LYMPHOCYTICLEUKAEYIA: A REPORT OF THREE CASES. C. Rulz-Marcellhn’, D. T. Vallespl, M. Torrabadella, Irrlguible. A. JaBn, and M. Saris’. Departments of Haematology and (‘1 Pathology. Hospital “Vail d’Hebr6n”. 08036 Barcelona, Spaln. Most frequently described assoclarlons between lymphoid myelodyaplaat.Ic syndromes (MDS) and mallgnanclea Include plasma cell tumours and B- and T-cell lymphomae. We have observed three cases of MDS: 1 refractory anaemla (RA). 1 RA wlth ring alderoblasta (RAS), and 1 RA wlth excess of blasts with (RAEB) coexletlng chronic lymphocytlc leukaemla (CLL) of B lineage (CDlQ+, CD6+, SmIg+ cells). In two cases both dlseases were diagnosed simultaneously. In one case, the patlent was prevlously dlagnosed of RA (Hb 66 g/L, WBC 4.1 x 100 /L, platelets 315x10e/L wlth an hypercellular bone marrow showlng dyserythropolesls, marked dysgranulopoiesis and dysmegakaryopolesls). In the study of bone marrow histology, besldes ofher MDS features, some lymphold aggregates were observed. In splte of sterold Twenty-slx months later, this patlent presented with a perlpheral therapy, blood picture typlcal of CLL (WBC 21.3xlOe/L. 42% Lymphocytes were CD19+ (7490 and lymphold cells). showed SmIg (96% weak posltlvlty). Retrospective etudles uslng monoclonal antlbodles (LN2, MB2 from Clonab. and anti-HLA-DR) agalnst antigens preserved In parafln-embedded samples proved the B nature of at the tlme of the RA lymphold aggregates, dlagnosls. These observations support that, In some cases, CLL may arise from plurlpotentlal stem cells.
SECONDARY
MYELODYSPLASTIC
LYMPHOPROLIFERATIVE
SYNDROME IN
DISEASES.
K.Tm&n$, P.Cieslar’, I SpiEka’, R.Neuwirtod’ and Cze hoslovak WS Cooperative Group; 4st Hed Dept, Dept HaePatol, s Dept Clin Hsematol, 1st Medical Faculty, Charles University, u neaaocnice 2, CS-12808 Prague, Czechoslovakia Several studies have shorn that aponoclonsl gammopathiesare nore frequent in myelodysplastic syndrome in comparison with controls. 36 cases of secondary HDSfolloved by CzechoslovakHDS
CooperativeGroup were studied. 14 cases (3S,9r) were preceeded by lymphoproliferative disease and in 11 (30,6l) cases out of themwas foti monoclonalgarmopathy. This group consists of 9 cases of plasrocytoaa (25,Ot) and 2 cases of non-liodgkin’s lymphoma- impunocytoma with monoklonal gallpopathy (5,6%). Diagnosis wasestablished in 10 cases as RA,in 2 as RASand in 2 as RUB. All patients were treated by chemotherapyand someby actinotherapy as well. Bowevertwo cases whereBIDS developedvery fastly during 3 months(after one cycle of Alkeran therapy) were observed. Transformationof HDSto acute leuktia was observed in 4 cases. The mean interval from diagnosis of monoclonal gammopathy to HDSwas 49 months. Solwinteresting data were found in this group in comparison to the remaining 22 patients with secondary NB9with other origin. 10 cases (66,78) of all RA patients and only 2 patients (14,39) of all RUBpatients were observed in this group. Hediansurvival after HDSdiagnosis was longer in this group with lymphoproliferative diseases (8 manths v. 6,5). Influence of immunological abnormalities on onset of secondaryHDSis discussed.
ANALYSIS OF 36 CASES OF MYELODYSPLASTIC SYNDROME
SECONDARY
K.Trn&I)il,P.Ciesla+, I.Qpifika’, RJeuwirtovi2 and Cz hoslovak “i Dspt Clin HDS- CooperativeGroup. 1st DeptHed, Dept Haematol, Haematol,1st Hedical Faculty, Charles University, U nelaocnice2, Cs-128OSPrague, Czechoslovakia CzechoslovakHDS- cooperative group followed 627 patients with HDStill 1990. Diagnosisof secondaryHDSwas established in 36 (5,7%) patients. RAwasobserved in 15 cases (41,7t), RASin 4 cases (ll,l!), RAF8in 14 (38,9#), RUB-t in 2 (5,6%)and CM in 1 (2,SO). There were 25 females and 11 males in the group, age medianwas 64 years. Professional exposure to X-ray irradiation was observed in 2 cases and to chemicals in 1 case in their previous history. Haematological neoplasia vas observed in 15 cases (14 cases of lymphoproliferative disease, 11 with rtonoclonalgamaopatby), aplastic anaemiain 2 cases, cancer in 13 cases, autoimmune disease in 2 cases, inflamtory disease in 1 case. The occurenceboth carcinoaa and secondaryWS (RAand RUB) after exposure to myelotoxic drugs and chenicals was observed in two cases. In previous history of these patients 13 were treated by myelosuppressivedrugs, 9 by actinotherapy, 7 by both measures, 4 by immunosuppressive therapy. Howeververy fast development of secondary WS after minimaldoses of cytotoxic drugs were observed in 3 cases. Pathological cytcgenetic findings were found in 90,5t cases, at least one typical abnormality (5VI-517(1-, -7) in 73,7#. Hedian interval betweenaetiological factor and onset of HDSwas 48 months, mediansurvival of patients with developedWS was 9 months.Transformationto acute leukaeaia wasobserved in 11 (30,6i) cases. Comparisonto data of the wholegroup of HDSis made.