AML, MPD Flashcards

1
Q

Mention the common findings in AML

A

1_Anemia: pallor fatigue weakness
2_thrombocytopenia: bleedind bruisisng.
3_fever that fails to respond to therapy.
4_splenomegaly, hepatomegaly, lympadenopathy
5_bone tenderness.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what are the hematological and bone marrow findings in AML

A

1_hema: normocytic normochromic anemia, occasionally macrocytic but don’t response to B12 or B9.
nucleated RBC, anisocytosis, poikilocytosis.
platlete count is moderatly depressed(hypogranular and giant).
WBC is variable.

BM: hypercellular, with blasts> 20% to deffrintiate it from MDS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Mention the FAB classification of AML

A

M0 A myeloblastic L minimaly defferntiated.
M1 AML without maturation
M2 AML with maturation
M2 baso(with basophil blasts)
M3 hypergranular promylelocytic L
M3 variant (hypogranular bilobed, micro).
M4 myelomonocytic L
M5 monocytic L (M5a poorly dif, M5b well deff).
M6 erythroleukemia
M7 megakaryocytic leukemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Explain some charecters of M0

A
  • most common in adult.
  • WBC: 40% leukocytosis,> 50% leukocytopenia.
  • diagnosis: if less than 3% of blasts are positive for peroxidase or SBB and myeloid markers: CD13,14,15,33,34 and negative for B, T cells markers.
  • BM is hypercellular with blasts
  • Aur rods are not found.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

explain some charecters of M1

A
  • predominant cells is blood are are poorly deff myeloblasts with finely reticulated chromatin.
  • Leukocytosis in> 50% of ptn.
  • Auer rods found in 50% of blasts, if not found it resemble L2 lymphoblast
  • positive peroxide and SBB in more than 3%
  • 50% of ptn have aquired clonal chromosomal abberrations. t(9:22).
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

explain some charecters of M2

A
  • most common subtype of AML
  • predominant cells are psudopleger Huet and hypogranular neutrophils
  • 50% leukocytosis
  • monocyte component is less than20% to deff from M4
  • high basophil and esinophilia
  • 50% have t(8:21)
  • better prognosis than average.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

explain some charecters of M3

A

-seen in younger patients
-most common finding: bleeding
-serious complications: DIC
-80-100% have t(15:17)
- unique treatment (all trans retinoic acid).
- two forms: typical hypergranular
variant hypogranular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the special characters of M4

A
  • have myeloid and moncytic characters
  • elevated serum/urea muramidase
  • high frequency of gum(hyperplasis, bleeding) , skin, manengieal involvment.
  • poor prognosis
  • positive for SBB, peroxidase, specific and non specific estrase
  • peripheral blood: monocytes> 5000
  • BM. monocytes> 20%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the special characters of M5

Schilling

A
  • high frequency of extramedullary infiltration of lungs, colon, menegies, LN, bladder, larynx
  • gum hyperplasia
  • S/U muramidase is extremely high
  • one criterion for diagnosis is 80% or more of all nonerythroid cells in BM are monocytes.
  • two forms: a; poorly deff (maturations index <4%) , B; maturation index> 4%).
  • M5a: granulocytes <20%, monocytes> 80%,monoblast > 80%.
  • M5b: granulocyte <20%,monocytes > 80%, monoblasts <80%.
  • peroxidase and SBB are weak
  • positive NSE, ANE both substrates.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the special characters of M6

DiGuglieumo

A
  • rare type that primerly affect peripheral cells.
  • nonexist in children
  • MDS often preceed it(erythemic myelosis).
  • most common presentation: bleeding.
  • most common change in blood: anemia with anisopoikilocytosis, nucleated rbc,sideroblast.
  • platlets, WBC usually decreased
  • psitive pas
  • CD 41,42,61
  • abnormalities in chromosome 21
  • diagnosed when> 50 % of all BM cells are erythroid, 30% of remaining are blasts
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are the special characters of M 7

A
  • rare, occurs as trasformation of MDS and CGL
  • bone marrow dry tap is common
  • Anemia and pancytopenia is characteristic
  • peripheral blood: megakaryocytes, undeff blasts
  • BM biobsy: increased fibroblast +reticulin and> 30% blasts
  • negative peroxidase, PAS(-,+), Estrase(+,-) , positive acid phosphatase.
  • positive Alpha N acetate estrase, but negative Alpha N butyrate estrase.
  • CD 41,42,61, AB against platelete glycoprotein 1b,2b,3b,3a
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

mention the complications of AML

A

1_infection
2_bleeding, DIC
3_tumer lysis syndrome
4_leukostasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Mention the causes of AML

A
1_radiation
2_chemical drugs «leukemogens» 
3_onchogens
4_protoonchogens
5_genetic factors
6_viruses«C RNA»
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Do you memorize the table of Cytochemical differntiation of acute leukemia?

A

subtype.MPO.SBB. PAS. ANAE. AP
M0; + + - - -
M1,2,. +++ ++ + - - +m2
M4. -/++ +/++ - + +
M5. -/+ -/+ - +++ +
M6. - - + + - +
M7. - - - - -
BALL. - - ++ - -
TALL. - - - - +

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Regarding polycythemia vera:
A_clinical features and complications.
B_lab findings.
C_critria to diagnose

A

A_
1_plethooric appearance, iching after hot shower
2_symptoms of slow circulation (cardia, neuro, GIT)
3_symptoms of increased metabolism(sweating, weigt loss)
4_bleeding tendency
5_HSM
6_Complications: CML, myelosclerosis, RF, thrombosis, gout, peptic ulcer.
B:
1_increased Hb, HC, blood viscosity
2_low ESR
3_High WBC C with slight shift«metamyelocyte», H basophils
4_plat c high, decrease level of PF3
5_BM, hypercellular, hyperplasia of erythroid series, Low iron stores, high reticulin.
6_LAP high
7biochemstry: high B12 and B12 binding ptn, low erythropoitein, ferretin, folate.
hyperurecemia and H LDH.
C

1_Jak 2+: Hct> 52% m,> 48% female.
2_Jak2-: major: Hct> 60% m,> 56%f
no secondary erythrocytosis.
palbable splenomegaly, aquired genetic abnormalities«except Jak, BCR-ABL».
minor:
plat> 450×106
Neutrophils> 10 ×106
radiographic splenomegaly
low serum erythropoitein
*4major criteria or 3 major+2minor for diagnosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Regarding IM:
1_cause
2_clinical features
3_lab findings

A

1_primary: idiopathic and un common
secondary: infiltration by malignancy or infection or chemicals or irradiation.
2_progressive anemia, splenomegaly,hepatomegaly, fibrosis.
bleeding due to dysmegakaryocytopoisis is the most constant feature.
osteosclerosis, bone pain, malaise.
3_leukocytosis with leukoerythroblastic picture, tear drop cells, NRBCs, immature myloid cells.
BM is hypercellular and fibrotic «dry tap».

17
Q
Regarding ET: 
1_how to diagnose it
2_most common diorders in patients 
3_lab findings
4_male: female ratio
5_plat function
A

1_by exclusion of other MPD and non hematological illness associated with increased plat count.
2_bleeding or thrombotic problems
3_high platlete c; exceed 1million, with a minimum of 6 m.
Hb low, hypochromic microcytic anemia, with splenic atrophy«target cells, HJ bodies, NRBCS, acanthocytes.
WBC C increased in 50%.
ALP N or H
B12 and uric acid increased.
BM: hypercellular, megakaryocytic hyperplasia, polypoid of the nuclei, giant forms and clusters.
4_equal
5_mean extent of aggregation is lower than normal.

18
Q

memorize the table of differential diagnosis of MPD

A

Gallery

19
Q

Mention the clinical features of CML

A
1_minimal intensity symptoms, fatigue, anorexia, weigt loss, abdominal discomfort. 
2_pain due to splenic infarction 
3_hemorrhage 
4_gout, visual disturbance 
5_neuro abnormalities (piriapism).
20
Q

when dose ANP value change?

A

1_increase in: PV, ET, A leukemia,leukemoid reaction,carcinoma, MM,HL,osteomyelosclerosis,prenicous anemia,sepsis,sarcoidosis, fever secondary to infections.
2_decrease or ansent: congenital hypophosphatase, CML, PNH, sideranemia, rheumatic disease

21
Q

What are the lab findings in CML

A

1_moderate anemia «normocyticnormochromic»… severe later.
2_leukocytosis up to 500k,mainly «segmented neutrophils, myelocytes comprise 10-50%»
3_increased lymphocytes, monocytes.
4_plat normal or increase
5_hyperurecemia, hyperuricosuria, H LDH, H serum K, NAP < 6%.
6_BM: hypercellular, mainly myeloid series, erythropoiesis is normal, somtimes dyserythrotic.
7_M: E ration increased
8_megakaryocyte are prominent and usually smaller than normal.

22
Q

What are the stages of CML

A

1_ Chronic phase: blood;
GP: shift to left, psudopleger, esinophilia, basophilia.
EP: Normoblasts, anisocytosis.
THP: increase plat, immature, anisocytosis.
BM: GP: hyperplasia, shift, esino baso
EP: decreased
THP: increased with normal form.

2_Accelerated phase: Blood: 
GP: shift, blast<20%, basophils<30%
EP: normo, anisocytosis. 
THP:normal or decrease anisocytosis.
BM: GP:shift,blast 15_20%, basophilia
EP: D.          THP: normal or D. 
3_Blast crisis: Blood 
GP: blast increase 
EP: anisocytosis, polychromesia, normoblasts
THP:decrease or absent, anisocytosis. 
BM: GP: blast> 30%
EP: D.         THP: D