EBH tutored case 14. Prof Dr Irene Lorand-Metze State University of Campinas

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1 EBH tutored case 14 Prof Dr Irene Lorand-Metze State University of Campinas

2 History and physical examination Male 49 years old patient. Since 2005 he had arterial hypertension. Renal insuficiency. He smoked around 30 cigarettes a day. He was referred to the hematologist in january 2007, when a pancytopenia was found in one of his periodic PB counts. Physical examination: pale mucosa ++. Blood pressure: 165 x 95 mmhg No other alterations were found.

3 Peripheral blood counts 15/01/2009 Hb : 7.4 g/dl MCV: 5 fl MCH: 36 pg Platelets: 91 x 9 /l Leukocytes: 2.8 x 9 /l neutrophils: 1.1 x 9 /l

4 Bone marrow cytology 20/01/2009 Erythroblasts: 37% Granulocytes: 55% Blasts 7.5%, Promyelocytes 8.5%, Myelocytes 8.5% Atypical megakaryocytes

5 Bone marrow biopsy and cytogentics Hypocellular marrow with some cellular foci Karyotype: 45XY, -7

6 Diagnosis Peripheral blood counts and bone marrow cytology lead to the diagnosis of MDS. WHO type: RAEB, based on cytology. Karyotype : high risk IPSS: high risk

7 Revisão do risco citogenético Schanz et al 2011 muito bom: del(11q), -Y bom: normal, del (5q), del (12p), del (20q), duas anormalidades incluindo del(5q) intermediária: -7/7q, +8, i(17q), -19, +21, qualquer outra alteração com 1 anormalidade, qualquer outra alteração com 2 anormalidades, clones independentes alto (3 anormalidades, 2 anormalidades incluindo -7/7q, der(3)(q21)/der(3)(q26) muito alto (mais do que 3 anormalidades)

8 Novo score IPSS (IPSS-R) Cariótipo peso 8 % blastos MO peso 6 Hb peso 2 Leucócitos peso 1 Plaquetas peso 1 5 grupos prognósticos

9 Table 1. Revised IPSS: Clinical risk groups and outcomes for MDS patients Greenberg et al 2011 Very Low Low Intermediate High Very High median survival 6.8 years median survival 4.3 years median survival 2.3 years median survival 1.5 years median survival 0.9 years

10 immunophenotyping Granulocytes with a low SSC Monocytes: 1.3% abnormal CD13 in maturing Gran CD45 PerCP -> Cells in the blast gate: CD34+ cells 6.3% CD34/CD19+ cells 0% CD34/CD13+ cells 3.94% CD34/CD13- cells 1.1% Blast index: 0.63

11 Análise das células CD34+ e maturação granulocítica CD34 PE -> CD19 FITC -> CD16 FITC -> Maturação anormal de granulócitos CD34 PE -> CD13 APC ->

12 Evolution of hematological parameters Peripheral blood 11/7/2007 Hb: 8.4g/dl, leuko.: 1.8 thromb.:57 9/8/2008 Hb: 7.2g/dl, leuko.: 2.9 thromb.:93 14/11/2008 Hb: 8.8g/dl, leuko.: 2.3 thromb.:77 Bone marrow blasts 15% 6.5% 8.5%

13 How to define blasts? Which variable has more prognostic relevance? Blasts, CD34+ cells or karyotype?

14 What factors do influence blast counts? Number of cells counted (500 cells recommended) Sample dilution (cytology x biopsy) For cytometry (vol 1mL) BM fibrosis Abnormal expression of CD34 (leukemia and MDS) what is more important for prognosis: blasts in cytology, total CD34+, abnormal coexpressions in CD34+ or % very early precursors?

15 Valor prognóstico de hemograma, MO citometria e índices Reis-Alves et al 2011 Hb (-) p = % Bl MO (+) p < CD34+ (+) p < CD34+/CD13- (+) p = Nr alterações (+) p < IPSS (+) p < OMS (+) p = Multivariada Hb (-) p = 0.07 % Bl MO (+) p = Nr alterações fenotípicas (+) p < 0.04

16 Follow-up Peripheral blood values remained stable, although transfusion dependency increased. Karyotype remained unchanged The patient is young and has a HLA-matched sibling. Should he receive a transplant? His HCT-CI is 3

17 Co-morbidades que influem no resultado do TMO alo Sorror et al Blood ponto: arritmia cardíaca, d. coronariana, ICC, infarto, diabetes: não complicado AVC prévio,d. psiquiátricas, hepatite, IMC >35. 2 pontos: d. reumatológicas, crea >2mg, DPOC leve 3 pontos: neoplasia pregressa, valvulopatia cardíaca, DPOC severo, cirrose hepática.

18 Sobrevida pós-tmo de acordo com o risco Sorror et al Blood 2005 NRM 2 anos Sobrevida 2 anos: Score: 0: 14% 1 a 2: 21% 3 ou mais: 41% Score 0: 71% 1 a 2 : 60% 3 ou mais: 34%

19 Treatment options for the patient Chemotherapy x hypomethylating agents (HA): Cht. gives a higher rate of CR, but a lower OS. More benefit of HA in patients with high risk cytogenetics Side effects: myelosupression in the initial cycles BMT: RIC is better than conventional due to the age and co-morbidities of patients HCT-CI 3 has a 2 year NRM of 41% Itzykson and Fenaux 2009, Sorror et al 2005, Greenberg 20

22 Reference values Normal BM Reis-Alves SC et al 2009, 20 % cel gate blastos : 1.26% ( ) % CD34+ : 0.78 ( ) % CD34+/CD13+ : 0.31 ( ) % CD34+/CD117+ : 0.21 ( ) CD34+/CD13 - : 0.2 (0 0.5) Precurs.B (CD34+/CD19+) : 0.14 ( ) Monócitos: 2.1% ( ) Monócitos CD16+ : 0.23 ( )

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