Tricoleucemiacom Alterações Psiquiátricas?

Uma breve revisão

Francisco Vilaça Lopes vilaca.lopes@gmail.com Médico Interno do 3º Ano de Medicina Geral e FamiliarEstágio de Saúde Mental ; Reunião do Serviço de Psiquiatria do Hospital do Barlavento Algarvio

Portimão, Algarve, Portugal, 2 de Outubro de 2015

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

Factores de risco

Fortes:● Meia-idade● Masculino● Caucasiana● Hemisfério ocid.

Fracos:● Benzeno,

organofosforados, radiação, serragem

● Genética● Vírus de Epstein-Barr

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

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 nosufficient data on treatment of hairy cell variant.

Hairy cell variant is characteristically CD25- CD123-, annexin A1-. This helps todistinguish the variant form from classical HCL.

Monocytopenia ischaracteristic.

.

Hepatitis B testing is indicated because of the risk of reactivation withimmunotherapy + chemotherapy. Tests include hepatitis B surface antigen andcore antibody for a patient with no risk factors. For patients with risk factors orprevious history of hepatitis B, add e-antigen. If positive, check viral load andconsult 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 MatureB-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

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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

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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

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b

c,d

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IHC panel: CD20, CD25, CD123, cyclin D1or

Annexin A1

BRAF

BRAF

See InitialTreatment (HCL-2)

NCCN Guidelines Version 4.2014Hairy Cell Leukemia

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 4.2014Hairy Cell Leukemia

�

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Cladribine

Pentostatin

f

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Clinical trial

Alternate purine analog

Interferon alpha

±

rituximab

Rituximab alone

INDICATION FOR

TREATMENT

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Systemic symptoms

Splenic discomfort

Recurrent infection

Hemoglobin <12 g/dL

Platelets <100,000/mcL

ANC <1000/mcL

No

indicationObserve

Indication

present

Observe until

indication for

treatment

RELAPSE/

REFRACTORYh

Relapse at

1 year�

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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 morphologicexamination of bone marrow biopsy or peripheral blood samples, resolution of organomegaly by physical e sence of disease symptoms. Eradication ofminimal 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

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!