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Hematalogy Lecture > Unit 3: Myelodysplastic Disorders > Flashcards

Unit 3: Myelodysplastic Disorders Flashcards

1
Q

Three Groups of Clonal Myeloproliferative Disorders:

A
  1. AMLs
  2. Chronic Myeloproliferative Disorders (CMPDs)
  3. Myelodysplastic Syndromes (MDSs)
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2
Q

MDSs are typically seen in what population?

A

in older (>50 years) pts., especially males

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3
Q

What are the 3 types of diseases of MDSs?

A
  1. Primary (de novo)
  2. Treatment-related (2-5 yrs. post-chemo.)
  3. Very rarely, inherited (FYI: There is a pedi. MDS in this category.
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4
Q

Myelodysplastic Syndromes (MDSs):

Often called preleukemias: they range from a mild anemia to a malignant disease that rapidly terminates in _____!

A

AML

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5
Q

Difficult disease to recognize for what it is. Incidence of MDS cases wasn’t even tracked until _____.

A

2001

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6
Q

MDS general characteristics:

Apparently the stem cell DNA sequences acquire permanent mutations, particularly involving chromosomes ___ and ____.

  • Most cases involve a defect at the Myeloid Stem Cell
  • Each mutated stem cell produces a unique clone w/ a specific cellular
    defect….leading to multiple expressions!
A

5 and 7

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7
Q

MDS:

Apoptosis is _________ early in disease, but ___________ later when converting to leukemia

A

increased

decreased

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8
Q

MDS general characteristics:

Result is ineffective clonal _____________ ( = too few cells) plus morphologic
__________ (= “ugly” cells)!

A

hematopoiesis

dysplasia

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9
Q
  • MDS cells apparently produce cytokines that kill off N. cells, so . .
  • Progressive p.b. __________ occur, with dyspoiesis (abnormal development)
    in 1 or more cell lines
A

cytopenias

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10
Q

All MDSs have…

______________– occurs first; see oval macrocytes (1 st thought? Megaloblastic anemia), megaloblastoid bone marrow with bizarre nRBCs, some ringed sideroblasts, & dimorphic RBCs

A

Dyserythropoiesis

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11
Q

Most MDSs have…

________________ - persistent basophilic cytoplasm, abnormal granules & nuclei, uneven cytoplasmic staining, & occ’l. Auer rods (even before it’s officially a leukemia).

A

Dysmyelopoiesis

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12
Q

Some MDSs have…

____________ - giant plts., bizarre & mononuclear megakaryocytes, & abnormal granules

A

Dysmegakaryopoiesis

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13
Q

What is a major feature of MDS?

what is NOT seen until disease transforms into leukemia?

A

*cytopenias

hyperproliferation

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14
Q

MDS can stay stable for years with only ________ evident.

A

dysplasia

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15
Q

Pts may be _________ to treatment once therapy is finally needed.

A

refractory

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16
Q

Do cells have normal function with MDS?

A

no, abnormal.

-these conditions have variable severity

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17
Q

MDS clinical symptoms

A
  • Fatigue
  • Fever
  • Bleeding

-However, many pts. are asymptomatic in early stages.

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18
Q

General P.B. Findings in MDSs

A
  1. Progressive cytopenia
  2. Megaloblastoid dyserythropoiesis in bone marrow
  3. Neutropenia
  4. Giant plts., circulating micromegakaryocytes/-blasts! (Similar to AMegL)
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19
Q

Megaloblastoid dyserythropoiesis in bone marrow, which manifests in p.b. as…

A

Hgb <10 g/dL
Variable degrees of : aniso, poik, Howell-Jolly bodies, basophilic stippling, & bizarre nRBCs

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20
Q

MDS P.B findings with neutropenia?

A

↑ metas & myelos, with morphologic abnormalities; may be Auer rods, may exhibit pseudo-Pelger-Huet

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21
Q

General Bone Marrow Findings in MDSs?

A
  • Bone marrow sample always required for diagnosis.
  • Bone marrow generally always hypercellular due to
    erythroid hyperplasia.
  • Ringed sideroblasts common.
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22
Q

General Bone Marrow Findings in MDSs:

Hallmark finding is…

A

***megaloblastoid dyserythropoiesis!!!

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23
Q

FAB Classification Scheme for MDSs relies on _____________ alone, so has limited prognostic utility.

A

morphology

-Each of these is pretty much a basic Refractory Anemia (RA), but with increasingly more severe problems overlaid on top of it

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24
Q

In order of increasing severity (some pts. will actually progress through each until it finally becomes an AML!):

A
  • Refractory Anemia (RA)
  • Refractory Anemia w/ringed sideroblasts (RARS)
  • Refractory Anemia w/excess blasts (RAEB)
  • Chronic Myelomonocytic Leukemia (CMML)
  • Refractory Anemia w/Excess Blasts in Transformation (RAEBIT, or RAEB-t)
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25
Q

FAB subgroups of MDS (general criteria)

A

*% blasts in bone marrow
*% blasts in p.b.
*± ringed sideroblasts
*% of monocytes
*extent of cytopenias
*degree of dyspoiesis (bizarre forms)

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26
Q

Four most common WHO subgroups of MDS

A
  1. Refractory Anemia (RA) - Refractory Cytopenia with
    Unilineage Dysplasia (RC)
  2. Refractory Anemia w/ringed sideroblasts (RARS)
  3. Refractory Anemia w/excess blasts (RAEB)
  4. Condition called MDS Unclassified (MDS-U).
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27
Q

Refractory Anemia (RA) - Refractory Cytopenia with
Unilineage Dysplasia (RC):

stable deletion syndrome (found in elderly women, has good prognosis.)

A

5q- Syndrome

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28
Q

Refractory Anemia (RA) - Refractory Cytopenia with
Unilineage Dysplasia (RC):

(like RARS, but affects > 1 cell line.)

A

Refractory PanCytopenia with Multi-lineage Dysplasia,
or RCMD

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29
Q

Chronic Myelomonocytic Leukemia (CMML) – moved to what classification?

A

myeloproliferative
disorders as MPN, Unclassified

30
Q

Refractory Anemia w/Excess Blasts in Transformation – moved to what classification?

A

AML

31
Q

Primary finding with Refractory Cytopenia with
Unilineage Dysplasia?

A

anemia with oval macrocytes (but not a simple ovalocytosis – this is a true
megaloblastoid picture)

32
Q

Refractory Cytopenia with
Unilineage Dysplasia:

-bone marrow blasts ___%.
-Rare ringed sideroblasts (_____%) & siderocytes.

A

<5

<15

33
Q

Refractory Cytopenia with
Unilineage Dysplasia:

  • % p.b. blasts _____%.
  • Normal B 12 & folate levels.
A

<1

34
Q

Is Dysmyelopoiesis or dysmegakaryopoiesis present with Refractory Cytopenia with
Unilineage Dysplasia?

A

rarely present

35
Q

Just like RA, but with more ringed sideroblasts (>15% of nucleated cells in bone marrow):
* < 5% blasts in bone marrow
* Usually exhibits dimorphic RBC population

A

Refractory Anemia with Ringed Sideroblasts (RARS)

36
Q

RA, + cytopenia in 2 cell lines now, & even more blasts!

A

Refractory Anemia with Excess Blasts (RAEB)

37
Q
  • Advent of prominent dysgranulopoiesis, plus some dyserythropoiesis (& rarely, some dysmegakaryopoiesis.)
  • May have pseudo-Pelger-Huet.
A

Refractory Anemia with Excess Blasts (RAEB)

38
Q

Refractory Anemia with Excess Blasts (RAEB):

  • ___% blasts in p.b.
  • Bone marrow blasts now up to _____%!
A

less than or equal to 5

5 - 20

39
Q

Refractory Anemia with Excess Blasts (RAEB) symptoms?

A

anemia, fever, bleeding, and infection

40
Q

RA, but with mostly monos in bone marrow
(>20%)

A

Chronic Myelomonocytic Leukemia (CMML)

-This is NOT the M4 AMML!

41
Q

Chronic Myelomonocytic Leukemia (CMML) is unusual MDS bc. it’s only one to have leukocytosis instead of ?

A

leukopenia

42
Q

WHO has moved Chronic Myelomonocytic Leukemia (CMML) into what category?

A

Myeloproliferative Disorders category

43
Q

Chronic Myelomonocytic Leukemia (CMML):

Absolute p.b. _____________ with morphological abnormalities always present

A

monocytosis

44
Q

Chronic Myelomonocytic Leukemia (CMML):

still ___% blasts in p.b.

A

<5

45
Q

What bone marrow stain/s would we do for monos?

A

NSEs

46
Q

What bone marrow stain would we do for myelocytes?

A

MPO and SBB (and maybe SE)

47
Q

CMML:

The assay for what substance would you expect to be elevated in serum and urine?

A

lysozyme

48
Q

Total WBC count with CMML?

A

increased!

49
Q

Monos in bone marrow with CMML?

A

at least 20%

50
Q

Total WBC count with RC, RARS, and RAFB?

A

normal to decreased

51
Q

Monos in P.B. (AMC) with CMML?

A

> 1 x 10^3/uL

52
Q

Blasts in bone marrow is _____% with RC and RARS.

A

<5

53
Q

Blasts in bone marrow is ______ % with RAEB and CMML.

A

5-20

54
Q

Dyserythropoiesis is seen with…

A

RC (especially) and RARS

55
Q

Dysgranulopoisis is seen with…

A

RAEB, CMML

56
Q

What disorders are Siderocytes/Sideroblasts seen with?

A

RC and RARS

+/- for RAEB

neg. for CMML

57
Q

Dysmegakaryocytopiesis may be seen with…

A

RAEB or CMML

neg for RC and RARS

58
Q

Ringed Sideroblasts in bone marrow is seen with…

A

RARs >15%

+/- for RC and RAEB

neg for CMML

59
Q

Generalized Lab Findings for MDSs:

Pos. for Fe stains for bone marrow sideroblasts, like…

A

Prussian blue stain

60
Q

Generalized Lab Findings for MDSs:

MPO/SBB and NSE stains all ________.

A

positive

61
Q

Generalized Lab Findings for MDSs:

B12 and folate levels are ___________.

A

normal

62
Q

Generalized Lab Findings for MDSs:

Most common abnormalities involve what chromosomes?

A

5, 7, 8, 11, 13, and 20

-Trisomy 8 and monosomy 7

63
Q

Generalized Lab Findings for MDSs:

True or false?

genetic translocations are common.

A

False, rare!

64
Q

MDS median survival varies from ____ to ____ years.

A

less than one year to greater than 6 years

65
Q

MDS Prognosis & Treatment:

RC & RARS do better than ___________.

A

RAEB & RAEB-t

66
Q

MDS:

Pts. can die from what complications? (not just from transforming into AML)

A

anemia, thrombocytopenia, OR neutropenia

67
Q

MDS therapy depends on…

A

age, clinical severity, and cytogenetic picture

68
Q

Only cure for MDS?

A

bone marrow transplant but BRMs (Biologic Response Modifiers) under investigation.

69
Q

What does RC generally look like?

A

oval macrocytosis and nRBCs ie, megaloblastic old picture, with normal B12 and folate levels

70
Q

What does RARS generally look like?

A

RA, + ringed sideroblasts in b.m.; RBCs look dimorphic

71
Q

What does RAEB generally look like?

A

RA, + increased b.m. blasts, plus cytopenia in 2 cell lines, ANA bizarre WBCs!

72
Q

What does CMML generally look like?

A

RA, +monos, AND blasts. AND LOTS of bizarre WBCs!
NSE 4+!

Hematalogy Lecture (35 decks)

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