Tricoleucemia
com Alterações Psiquiátricas?
Uma breve revisão
Francisco Vilaça Lopes vilaca.lopes@gmail.com Médico Interno...
Definição
●
Neoplasia incomum de células
B maduras.
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Descrita como entidade clínica
distinta em 1958
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Ao microscópio, as...
Factores de risco
Fortes:
●
Meia-idade
●
Masculino
●
Caucasiana
●
Hemisfério ocid.
Fracos:
●
Benzeno,
organofosforados,
ra...
Clínica – Laboratório
●
Desconforto / plenitude abdominal
●
Fraqueza e fadiga
●
Palidez e petéquias
●
Febre
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Infecções op...
Version 4.2014, 08/22/14 © National Comprehensive Cancer Network, Inc. 2014, All rights reserved. The NCCN Guidelines and ...
Version 4.2014, 08/22/14 © National Comprehensive Cancer Network, Inc. 2014, All rights reserved. The NCCN Guidelines and ...
Bibliografia
http://portugal.bestpractice.bmj.com/best-practice/monograph/890/highlights.html
http://www.nccn.org/professi...
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Tricoleucemia com alterações psiquiátricas?

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Francisco Vilaça Lopes. Tricoleucemia com alterações psiquiátricas? [discussão]. Reunião do Serviço de Psiquiatria do Hospital do Barlavento Algarvio: Portimão, Biblioteca do Serviço, 2-10-2015. Diapositivos em http://bit.ly/1hgffRG

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Tricoleucemia com alterações psiquiátricas?

  1. 1. Tricoleucemia com Alterações Psiquiátricas? Uma breve revisão Francisco Vilaça Lopes vilaca.lopes@gmail.com Médico Interno do 3º Ano de Medicina Geral e Familiar Estágio de Saúde Mental ; Reunião do Serviço de Psiquiatria do Hospital do Barlavento Algarvio Portimão, Algarve, Portugal, 2 de Outubro de 2015
  2. 2. Definição ● Neoplasia incomum de células B maduras. ● Descrita como entidade clínica distinta em 1958 ● Ao microscópio, as células têm projeções citoplasmáticas delicadas, parecidas com cabelos. ● Leucemia/Linfoma não-Hogkin. ● +vo mutações BRAF V600E
  3. 3. Factores de risco Fortes: ● Meia-idade ● Masculino ● Caucasiana ● Hemisfério ocid. Fracos: ● Benzeno, organofosforados, radiação, serragem ● Genética ● Vírus de Epstein-Barr
  4. 4. Clínica – Laboratório ● Desconforto / plenitude abdominal ● Fraqueza e fadiga ● Palidez e petéquias ● Febre ● Infecções oportunistas recorrentes (P. carinii, Legionella, toxoplasmose, listeriose, etc.) ● Achados neurológicos (s. Guillain- Barré, sinais de meningite e compressão nervosa) ● Autoimunidade (poliarterite nodosa, pioderma gangrenoso, esclerodermia, polimiosite, maculopápulas eritematosas) ● Esplenomegalia ● Hepatomegalia ● Linfadenopatia superficial e profunda ● Anemia ● Trombocitopénia ● Neutropénia ● Dx citogenético
  5. 5. Version 4.2014, 08/22/14 © National Comprehensive Cancer Network, Inc. 2014, All rights reserved. The NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN . ®® NCCN Guidelines Index NHL Table of Contents Discussion Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. HCL-1 a b d e This guideline applies to hairy cell leukemia, not hairy cell variant. There are no sufficient data on treatment of hairy cell variant. Hairy cell variant is characteristically CD25- CD123-, annexin A1-. This helps to distinguish the variant form from classical HCL. Monocytopenia is characteristic. . Hepatitis B testing is indicated because of the risk of reactivation with immunotherapy + chemotherapy. Tests include hepatitis B surface antigen and core antibody for a patient with no risk factors. For patients with risk factors or previous history of hepatitis B, add e-antigen. If positive, check viral load and consult with gastroenterologist. cTypical immunophenotype: CD5-, CD10-, CD11c+, CD20+ (bright), CD22+, CD25+, CD103+, CD123+, cyclin D1+, annexin A1+. See Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and Neoplasms (NHODG-A)NK/T-Cell DIAGNOSISa WORKUP ESSENTIAL: Physical exam: Presence of enlarged spleen and/or liver; presence of peripheral lymphadenopathy (uncommon) Performance status CBC, differential, platelets Bone marrow biopsy ± aspirate USEFUL UNDER CERTAIN CIRCUMSTANCES Discussion of fertility issues and sperm banking · · · · · · · · · Peripheral blood examination Comprehensive metabolic panel with particular attention to renal function LDH Hepatitis B testing if rituximab contemplated Pregnancy testing in women of child-bearing age (if chemotherapy planned) Chest/abdominal/pelvic CT with contrast of diagnostic quality e · · ESSENTIAL: Presence of characteristic hairy cells upon morphologic examination of peripheral blood and characteristic infiltrate with increased reticulin in bone marrow biopsy samples. Dry tap is frequent. IHC and flow cytometry are essential for establishing the diagnosis and for distinguishing between hairy cell leukemia and hairy cell variant. Adequate immunophenotyping to establish diagnosis Cell surface marker analysis by flow cytometry: CD3, CD5, CD10, CD11c, CD19, CD20, CD22, CD25, CD103 USEFUL UNDER CERTAIN CIRCUMSTANCES: Molecular analysis to detect: IGHV mutational status Sequencing of for V600E mutation or IHC for mutant · · · · · · b c,d > > IHC panel: CD20, CD25, CD123, cyclin D1 or Annexin A1 BRAF BRAF See Initial Treatment (HCL-2) NCCN Guidelines Version 4.2014 Hairy Cell Leukemia
  6. 6. Version 4.2014, 08/22/14 © National Comprehensive Cancer Network, Inc. 2014, All rights reserved. The NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN . ®® NCCN Guidelines Index NHL Table of Contents Discussion Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. HCL-2 INITIAL TREATMENTh FOLLOW-UP NCCN Guidelines Version 4.2014 Hairy Cell Leukemia · · Cladribine Pentostatin f · · · · Clinical trial Alternate purine analog Interferon alpha ± rituximab Rituximab alone INDICATION FOR TREATMENT · · · · · · Systemic symptoms Splenic discomfort Recurrent infection Hemoglobin <12 g/dL Platelets <100,000/mcL ANC <1000/mcL No indication Observe Indication present Observe until indication for treatment RELAPSE/ REFRACTORYh Relapse at 1 year³ · · Retreat with initial purine analog rituximab Alternative purine analog rituximab ± ± Relapse at <1 year f g h Cladribine should not be administered to patients with active life-threatening or chronic infection. Complete response defined as: recovery of blood counts (Hgb >12 g/dL, ANC >1500/mcL, platelet >100,000/mcL), absence of HCL cells by morphologic examination of bone marrow biopsy or peripheral blood samples, resolution of organomegaly by physical e sence of disease symptoms. Eradication of minimal residual disease (as determined by flow cytometry, immunohistochemistry, or molecular analysis) is of unproven value at this point. . xam, and ab See Treatment References (HCL-A) < Complete responseg Complete responseg Adapted from: Grever MR. How I treat hairy cell leukemia. Blood 2010;115:21-28. Consider prophylaxis for tumor lysis syndrome ( ) See monoclonal antibody and viral reactivation ( ) See NHODG-B NHODG-B
  7. 7. Bibliografia http://portugal.bestpractice.bmj.com/best-practice/monograph/890/highlights.html http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf http://emedicine.medscape.com/article/200580-overview Muito obrigado!

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