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Wbc > Myeloid Disorders > Flashcards

Myeloid Disorders Flashcards

1
Q

What all come under the myeloid disorders

A

AML
Myeloproliferative neoplasms
Myeloblasts dysplastic disorders
Mpn / mds

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2
Q

Myeloproliferative disorders include

A

CML
Pcv
Essential thrombocytosis
Myelofibrosis

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3
Q

CML usually occurs in

A

Middle age and elderly

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4
Q

Clinical features of cml

A

Dragging pain due to splenomegaly

Hepatomegali

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5
Q

Which translocation occurs in cml

A

T 9 22occurs in the cml
Abl gene in the 9 th chromosome and bcl gene in the 22 chromosome fuses to form abl bcc gene
With high tyrosine activity
Auto activation occurs and cell proliferation occurs independently of growth factors

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6
Q

Treatment given in the cml is

A

Imatinib mesylTe

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7
Q

Imatinib messy late is also given in

A

Gist

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8
Q

Treatment of cml

A

Along with imatinib mesylate we give stem cell transplantation

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9
Q

Phases of cml

A

Chronic phase
Accelerated phase
Blast crisis

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10
Q

How can u differentiate blw chronic phase and accelerated phase

A 
In chronic phase the blast count is only <10% only 
In the accelerated phase lo 
Blasts are above >10%
Persistent thrombocytopenia 
Increasing tlc
Splenomegaly
Persistent thrombocytosis
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11
Q

What is blast crisis

A

Blast count
> 20%
Extra medullary blast prolifer

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12
Q

How can u diagnose cml

A

Cbp
Hb normals
Platers increase
Tlc increase

Pheripheral smear
College girl appearance
Rapidly dividing cells move from the bone marrow to the periphery
Promyelocytes myelocytes meta myelocytes all come to the pheripheral blood

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13
Q

What abou be aspirate findings in the cml

A

Myeloid and erythronium ratio
Dwarf megakaryocytes
Pseudo gaucherie cel
Sea blue histiocytes

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14
Q

In which conditions the nap score is increased

A 
In the myeloproliferative diseases except in the Cml
Infection 
Stress
Pregnancy 
Leukamoid reactions
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15
Q

In which conditions nap scores are reduced

A

In cml
Penh
Myelodysplastic syndrome

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16
Q

What is polycythemia Vera

A

Major criteria is
increase in the hb content more than 16 in females and males

Jak2 pathway mutation 
Hematocrit 49 males 48 females
Bm tri lineage 
Pleomorphism in megakaryocytes 
Minor 
Erythropoietin levels are low
17
Q

Most common pathway getting mutated is the pcv

18
Q

Clinical features of the pcv

A

Hb increase walla
staisis
Neurological abnormalilits

Bleeding disorders 
Itching
Erythropoietin less
Hepatosplenomegaly
Jak stat
19
Q

What is primary pcv and secondary pcv

A

Primary pcv lo erythropoietin levels are low
In the secondary they are high occurs in high altitude places
And in smokers and copd patients

20
Q

What is the treatment of pcv

A

Phlebotomy

21
Q

Essential thrombocytosis

A

Platelets count must be more than 4.5 lakh
CML exclude
Pcv exclude
Mpn exclude

Megakaryocytes hyperplasia stag horn apprenance occurs

Jak2 v617f. Mpl, calreticulin mutation any one can be seen

22
Q

Clinical features of essential thrombocytosis

A

Pain throbbing pain
Parasthesiss
Transient ischemic attack

23
Q

What is. Myelofibrosis

A

Here three major and 2 minor criteria is there
Either 3 major or 2 major and 1 minor need to be considered
Major
Atypical megakaryocytes
CML pcv mds mpn exclusion
Jak2v617f , mutation

Minor

Leh incre
Anemia
Splenomegaly
Leukoerythroblastosis

24
Q

Climb feature of myelofibrosis

A

Elderly

Splenomegaly

25
Q

Pathogenesis of myelo

A

Megakaryocytes hyperplasia valla pdgf increase which is fibrogenic amd fibrosis occur

26
Q

Lab diagnosis of myelofibrosis

A

Pancytopenia
Tear drop cells
Leukoerythroblastic blood picture

27
Q

Bone marrow aspiration of myelofibrosis

A 
Dry tap 
Also seen in 
Hairy cell leukaemia 
Myelofibrosis 
Metastatic cancer 
Amlm7

Bm biopsy reticulin fitted are seem

28
Q

What is langer Hans cell histiocytosis

A

Proliferation of immature dendritic cells

Brafv600e mutation

29
Q

What are pheripheral B cell neoplasm

A 
Cll or sll
B cell prolymphocytic lymphoma
Lymphoplasmocytic lymphoma
Splenic and nodal marginal zone lymphoma 
Extra nodal marginal zone lymphoma
Follicular lymphoma
Marginal zone lymphoma
Hairy cell leukaemia 
Plasma cell myeloma
Diffuse large cell lymphoma
Burkett lymphoma
30
Q

Chronic lymphocytic lymphoma / sll

A

Age 60-70 yrs
Most common
Del 13 q 11 q 17 p
13 q associated with micro 15 16 deletion

Btk gene mutation increase btk increase B cells increase antibodies

These can attack rbc and platelets

31
Q

What is evans syndrome

A

Cll + Aiha+ aithrombocytopenia

32
Q

Clinical features of cll

A

Fatigue
Painless lymph node enlargement
Hepatosplenomegaly

33
Q

Lab findings of cll

A 
Hb decrease 
Tlc= increase might
DLC  absolute lymphocytes more than 
Platelets = norma
l

Wbc (2 decks)

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