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HEMATOLOGIA > sindromes mieloproliferativas > Flashcards

sindromes mieloproliferativas Flashcards

1
Q

Caracterize as SMP

A

The MPNs are caused by acquired genetic defects in myeloid stem cells and are characterized by deregulated production of leukocytes, erythrocytes, or platelets. Although each disorder is named according to the dominant cell line affected, all can cause an elevation in several cell lines.

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2
Q

Cite caracteristicas em comum das SMP

A

Unusual thromboses, massive splenomegaly, or constitutional symptoms such as fever, chills, weight loss, and drenching night sweats

  • Progression to AML
  • Secondary fibrosis of the bone marrow
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3
Q

LMC

A

The clinical hallmark of CML is the uncontrolled production of mature and maturing granulocytes, predominantly neutrophils, but also basophils and eosinophils.
Granulocytic leukocytosis is present on CBC, and earlier myeloid forms such as metamyelocytes, myelocytes, and less than 5% blasts are present on peripheral blood smear review

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4
Q

Qual a mutacao genetica da LMC

A

acquired translocation of chromosomes 9 and 22 [t(9;22)] in an HSC, that leads to development of a fusion gene called BCR-ABL.

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5
Q

LMC trato quando

A

Trato ao diagnosticar ! - Inibidores da Tirosina kinase

Diferente da TE e Mielofibrose que podem ser observadas a priori

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6
Q

Ef colateral dos ITK

A

All TKIs can prolong the QT interval,

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7
Q

Cite um FR reconhecido para LMC

A

Exposicao a radiacao ionizante

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8
Q

O que é o hiato leucemico

A

A presenca de maior numero de mielocitos do que metamielocitos no SP dos pacientes com LMC

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9
Q

Leucocitos na LMC

A

São morfologicamente normais porem citoquimicamente alterados. FA neutrofilica está baixa o que faz a diferenca com Reacao leucemoide (aqui ha tb granulacao nos neutrofilos) e com PV (na PV a FA neutrofilica está alta)

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10
Q

Achados na LMC

A

Anemia normo normo
Trombocitose
Leucocitose com desvio para esquerda e <5% blastos em geral

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11
Q

Achados que definem fase acelerada na LMC

A

● 10 to 19 percent blasts in the peripheral blood or bone marrow
● Peripheral blood basophils ≥20 percent
● Platelets <100,000/microL, unrelated to therapy
● Platelets >1,000,000/microL, unresponsive to therapy
● Progressive splenomegaly and increasing white cell count, unresponsive to therapy
● Cytogenetic evolution (defined as the development of chromosomal abnormalities in addition to the Philadelphia chromosome)

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12
Q

TTO LMC

A
  • TMO se fase acelereda ou crise blastica
  • ITKs
  • Hidroxiureia
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13
Q

Caracterize Policitemia Vera

A

Desordem mieloproliferativa de precursores mieloides/ eritroides que gera proliferação de eritrócitos.
Aumento da massa de celulas vermelhas independente de EPO.

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14
Q

Mutação presente na PV

A

JAK 2

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15
Q

Marcos clínicos de PV

A 
Eritromelalgia
Prurido Aquagenico
Trombose venosa / AIT
Ex fisico> esplenomegalia e pletora
* EPO baixa, basofilia, leucocitose, trombocitose, JAK2+
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16
Q

Causas de eritrocitose secundarias que devem ser excluidas

A

Hipoxemia - DPOC, SAOS, Altitude
Neoplasias produtoras de EPO - Figado e rim
Cisto renais produtores de EPO
Uso de anabolizantes

17
Q

Como esta a VitB12, os basófilos e o Ac úrico na PV

A

Basophilia is a strong predictor of PV instead of a reactive state. Elevated serum vitamin B12 levels, caused by increased levels of transcobalamin III produced in proliferating leukocytes, and hyperuricemia as a consequence of DNA turnover in the marrow are classic for PV.

18
Q

O que é eritromelalgia

A

Dor em queimação nas palmas das mãos e pés com pulsos presentes e palidez/cianose/ eritema

19
Q

Trombose tipica da PV

A

Budd-Chiari

Também > esplênica e porta, mesenterica

20
Q

Sintomas de PV

A

Cefaleia, tontura, disturbios visuais, prurido, saciedade precoce e complicações - trombose e sangramento

21
Q

Achados laboratoriais na PV

A

Hb> 16,5 (49) homem e > 16 (48) na mulher
EPO normal ou baixa
Trombocitose e/ou leucocitose

22
Q

3 fases da PV

A

Latente
Proliferativa
e de Gasto (simula FMP)

23
Q

Trato PV quando?

A

No diagnóstico!
AAS + flebotomia em pacientes <60a sem trombose previa –> Ht<45%
Se >60a ou Evento trombotico previo -> acrescentar Hidroxiureia

24
Q

Paciente com declínio de necessidade de flebotomias e e Hidroxiureia + piora da esplenomegalia na PV

A

Sugere estar entrando na fase de gasto - Evolução para fibrose medular secundaria

25
Q

Dx de Trombocitemia essencial

A

> 600mil plaquetas em duas ocasioes com pelo menos um mes de intervalo
na ausencia de causas secundarias como anemia ferropriva

26
Q

Exs citogeneticos na TE

A

Mutacao JAK2

CALR

27
Q

Sintomas de TE

A

Digital ischemia, erythromelalgia, transient ischemic attack, visual disturbances, venous thromboembolism, or bleeding (due to dysfunctional platelets) may be present.

28
Q

Tratamento da TE

A

AAS,
Se alto risco -> Hidroxiureia se nao observar. Ex trombose >60a e Leuco>11mil
Plateletpheresis may be employed emergently to temporarily decrease platelet counts in symptomatic patients with extreme thrombocytosis (generally greater than 1,000,000/µL [1000 × 109/L]).

29
Q

Fibrose medular primaria

A

It is a clonal myeloid disorder characterized by abnormal, proliferating megakaryocytes that produce excess fibroblast growth factor. This causes marrow fibrosis and leads to extramedullary hematopoiesis.

30
Q

Aspirado de MO na FMP

A

Seco

31
Q

Achados no SP na FMP

A

Dacriocitos, hemacias nucleadas

32
Q

Sintomas tipicos de FMP

A

Febre, calafrios, sudorese noturna, fadiga. desconforto abdominal, saciedade precoce e perda de peso
Esplenomegalia , hepatomegalia (pode haver hipertensao portal)

33
Q

Indicações de Tto de FMP

A

Treatment is required for symptomatic splenomegaly, worsening cytopenias, and constitutional symptoms

34
Q

Tto de FMP

A

Hidroxiureia reduz a esplenomegalia e sintomas constitucionais; inibidor da JAK2 -> RIXOLITINIB

35
Q

Complicacoes da FMP

A

Complications include ascites, esophageal and gastric varices, gastrointestinal bleeding and hepatic encephalopathy.
Portal vein thrombosis is a recognized complication of PMF and other chronic myeloproliferative disorders [19] and may precede the clinical onset of the disease, similar to what has been documented in polycythemia vera

36
Q

Achados laboratoriais FMP

A

Anemia multifatorial
elevations in the serum concentrations of alkaline phosphatase, lactate dehydrogenase (hematopoiese ineficaz), uric acid, leukocyte alkaline phosphatase, and vitamin B12

HEMATOLOGIA (12 decks)

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