Self-evaluation Tutorial Prof Dr Irene Lorand-Metze State University of Campinas

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1 Self-evaluation Tutorial 2009 Prof Dr Irene Lorand-Metze State University of Campinas

2 SEV 3.2 start Male 34 years old patient. He came to the Emergency Unit of our Hospital referring high fever and cough for 3 days. Sputum was greenish. The patient suffered from schizophrenia and was using clozapine. Physical examination: he was in a bad physical condition. Blood pressure: 105 x 75 mmhg. Heart frequency: 98 beats/min Cefalexine had been instituted.

3 Peripheral blood counts Hb : 11.1 g/dl MCV: 85 fl MCH: 28 pg Platelets: 133 x 10 9 /l Leukocytes: 0.3 x 10 9 /l neutrophils: 0.1 x 10 9 /l

4 Avaliação clínica inicial Dados clínicos sugerem pneunonia lobar Hemograma: a série mais afetada é a granulocítica Hb e plaq. Excluem aplasia e leucemia aguda Até agora parece uma agranulocitose Nos antecedentes, tem medicação que pode dar agranulocitose (clozapina)

5 Question 1 According to the clinical data and the peripheral blood counts, which is the most probable diagnosis? 1. Agranulocytosis due to psychotropic agents and infection 2. Acute leukemia and pneumonia 3. Aplastic anemia and pneumonia 4. Myelodysplastic syndrome and infection 5. Fanconi s anemia and septicemia

6 Question 2 Which laboratory tests should be collected immediately? 1. Chest radiography 2. Bone marrow cytology and cytogenetics 3. Investigation of PNH 4. Bone marrow cytology and chest radiography 5. Bone marrow histology and DEB test Confirmar a pneumonia neutropenia mielograma

7 Question 3 - introduction The chest radiography revealed a pneumonia of the inferior lobe of the left lung. BM cytology showed: Granulocytic series with blasts and promyelocytes: 11% (Fig 1); erythroblasts: 58% (Fig 2); lymphocytes: 28%; megakaryocytes +++ (Fig 2)

8 Bone marrow cytology

9 Diagnóstico final Agranulocitose (por clozapina) e pneumonia lobar Para causas, ainda excluir: SMD cariótipo HPN pesquisa de deficiência de proteínas ancoradas GPI (CD55, CD59, CD66b, CD14) Anemia de Fanconi DEB test

10 Question 3 And now, which tests are necessary to conclude the diagnosis? 1. Karyotype 2. Investigation for PNH 3. DEB test All the above-mentioned tests

11 Question 4 Karyotype was normal. Expression of CD66b and CD14 on granulocytes or monocytes could not be performed due to the small number of cells in the peripheral blood. DEB test was negative. What is now the most probable final diagnosis? 1. Aplastic anemia 2. Myelodysplastic syndrome 3. Agranulocytosis due to clozapine 4. Acute leukemia 5. Fanconi s anemia

12 Question 5 - introduction After one month, the infection resolved and the patient was discharged. The psychiatrist changed clozapine to risperidone and DZP. PB counts: Hb : 8.8 g/dl; leukocytes: 5.7 x 10 9 /l; neutrophils: 3.1 x 10 9 /l; platelets: 100 x 10 9 /l

13 Bone marrow cytology BM: erythroblasts: 24%; granulocytes: 63% blasts: 0.5%. Megakaryocytes: +++

14 Question 5 What is the cause of the increased granula found in the granulocytic precursors? 1. a sign of persisting infection 2. due to the use of G-CSF 3. indicative of a myelodysplasia 4. due to BM recovery 5. a sign of myelotoxicity (psychotropic drugs)

15 Question 6 Why does the hemoglobin and the platelet did not fully recover? 1. The patient has aplastic anemia 2. Vitamin deficiency due to the use of parenteral nutrition 3. Myelotoxicity of the antibiotics used 4. the new psychotropic agents are also myelotoxic 5. The patient suffers from PNH. This could not be clarifyed during the infection and low granulocyte counts.

16 Anemia aplástica excluída: não houve pancitopenia desde o início. Os mielogramas são celulares HPN: não há dado outro dado clínico que sugere. Mas, se houver dúvida, pode-se repetir Deficiênica vitamínica: deveria ter sido dosado ácido fólico e B 12. Pode ocorrer em pacientes com sepse. Ver a nutrição parenteral e suplementar. A segunda citologia fala contra

17 Agranulocitose - causas Infecções Virais: HIV, dengue, hepatite, CMV Bacterianas: Gram tbc, febre tifoide Fungo: histoplasmose Protozoários: calazar, malária Autoimunidade d. Still, LED, etc Nutricionais - distúrbios alimentares, anemias carenciais

18 Medicamentos e agranulocitose Analgésicos: dipirona, indometacina, etc Psicotrópicos: clozapina, risperidona, diazepam, haloperidol Anticonvulsivantes Anti-tireoidianos (tiouracil) Antibióticos, drogas cardiovasculares (captopril, propanolol, nifedipina) etc

19 SEV conclusion Question 1-1 Question 2-4 Question 3-5 Question 4-3 Question 5-2 Question 6-4