Hye, 2014
LLC- INCIDÊNCIA
•
2 novos casos a cada 100000 pessoas/ano;
•
30% dos novos casos de leucemia;
•
Leucemia mais comum do mundo ocidental;
•
Quase exclusivamente devido à expansão clonal B;
•
Mais comuns em homens;
LLC-ETIOLOGIA
•
Desconhecida;
•
Teorias:
•
Genética;
•
Exposição viral (EBV, HIV) como no linfoma de Burkitt;
•
Altas doses de radiação: LLC-T;
•
Exposição a agentes tóxicos;
CLL= SINTOMAS
•
70% assintomático no momento do diagnóstico;
•
Sintomas comuns das leucemias:
• Febre; • Palidez; • Fadiga; • Respiração ofegante; • Sangramento fácil; • Infecções frequentes; • Sangramentos na gengiva; • Emagrecimento;
LLC- ACHADOS CLÍNICOS
•
Febre;
•
Esplenomegalia/hepatomegalia;
•
Linfoadenopatia;
•
Equimoses;
•
Sangramentos na gengiva;
•
Infecções inexplicáveis.
LLC- ACHADOS LABORATORIAIS
•
Hemograma com linfocitose e presença de sombras nucleares
de Gumpracht;
•
Células B CD19 e CD20 positivos;
LLC
Estágio
Risco
Achados Clínicos/Laboratoriais
0
baixo
Somente linfocitose
I
intermediário
Linfocitose com linfoadenopatia
II
intermediário
Linfocitose com linfoadenopatia,
esplenomegalia e hepatomegalia
III
alto
Linfocitose com anemia
IV
alto
Linfocitose com anemia e trombocitopenia
• Precursor B-cell neoplasm
• Precursor B-lymphoblastic leukemia/lymphoma
• Mature B-cell neoplasms
• Chronic lymphocytic leukemia/small lymphocytic lymphoma
• B-cell prolymphocytic leukemia
• Lymphoplasmacytic lymphoma
• Splenic marginal-zone B-cell lymphoma
• Hairy cell leukemia
• Plasma cell myeloma
• Monoclonal gammopathy of undetermined significance
• Solitary plasmacytoma of bone
• Extraosseous plasmacytoma
• Primary amyloidosis
• Heavy-chain diseases
• Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue
• Nodal marginal zone B-cell lymphoma
• Follicular lymphoma
• Mantle cell lymphoma
• Diffuse large B-cell lymphoma
• Mediastinal (thymic) large B-cell lymphoma
• Intravascular large B-cell lymphoma
• Primary effusion lymphoma
• Burkitt lymphoma/leukemia
WHO CLASSIFICATION OF
LYMPHOPROLIFERATIVE DISORDERS
B-CELL NEOPLASMS
PB morphology from a typical case of T-PLL showing medium-sized lymphoid cells with a regular nuclear outline, single nucleolus, and intense basophilic cytoplasm.
Dearden C Blood 2012;120:538-551
Galani KS, Subramanian P G, Gadage VS, Rahman K, Ashok Kumar M S, Shinde S, Mahadik S, Ansari R, Sengar M, Menon H, Nair R, Gujral S. Clinico-pathological profile of Hairy cell leukemia: Critical insights gained at a tertiary care cancer hospital. Indian J Pathol Microbiol 2012;55:61-5
Leucemia/linfoma T do adulto
WORLD HEALTH ORGANIZATION CLASSIFICATION
OF LYMPHOPROLIFERATIVE DISORDERS
• B-cell proliferations of uncertain malignant potential
• Lymphomatoid granulomatosis
•
Precursor T-cell
neoplasm
• Precursor T-lymphoblastic leukemia/lymphoma
•
Mature T-cell and NK-cell
neoplasms
• T-cell prolymphocytic leukemia
• T-cell large granular lymphocytic leukemia
• Aggressive NK-cell leukemia
• Adult T-cell leukemia/lymphoma • Extranodal NK-/T-cell
lymphoma, nasal type
• Enteropathy-type T-cell lymphoma
• Hepatosplenic T-cell lymphoma
• Subcutaneous panniculitis-like T-cell lymphoma
• Mycosis fungoides
• Sézary syndrome
• Primary cutaneous anaplastic large cell lymphoma
• Anaplastic large cell lymphoma
• Angioimmunoblastic T-cell lymphoma
• Peripheral T-cell lymphoma, unspecified
WHO CLASSIFICATION OF
LYMPHOPROLIFERATIVE DISORDERS
• T-cell proliferation of
uncertain malignant potential
• Lymphomatoid papulosis
• Neoplasm of uncertain
lineage and stage of differentiation
• Blastic NK-cell lymphoma
• Hodgkin lymphoma • Nodular lymphocyte predominance • Classical • Nodular sclerosis • Lymphocyte-rich • Mixed cellularity • Lymphocyte depletion • NK, natural killer.
WHO CLASSIFICATION OF
LYMPHOPROLIFERATIVE DISORDERS
• Wintrobe’s Atlas of Clinical Hematology _Adapted with permission from Wintrobe's Clinical Hematology, 11th Edition, page 2304.
