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Blood and lymph Unit II > Schowinsky MDS and MPN > Flashcards

Schowinsky MDS and MPN Flashcards

1
Q

Define myelodysplastic syndrome

A

group of conditions where the marrow is replaced by malignant clone, derived from a tranformed stem or progenitor cell

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2
Q

What are the 2 big characteristic features of MDS

A

ineffective hematopoiesis and increased risk of tansformation to acute leukemia

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3
Q

Clinical scenario for Primary (idiopathic) MDS

A

median age of diagnosis 70; usually over 50; 3-5 cases/100k/year

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4
Q

Clinical scenario for Secondary MDS

A

usually therapy related, occurs as part of spectrum of t-AML, usually diagnosed 2-8 years following therapy with DNA-alkylating agents or ionizing radiation. Contains complex karyotype with whole of partial deletions of chromosomes 5 and/pr 7

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5
Q

T or F: diagnosis of MDS is usually considered when persisten peripheral cytopenia in one or more lineages is present and cannot otherwise be explained

A

TRUE

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6
Q

T or F: If isolated persistent cytopenia is present, it is usually neutropenia or thrombocytopenia, not anemia

A

FALSE, other way around

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7
Q

With persistent cytopenia, what 3 modalities can be used to establish MDS

A

1) morphologic evidence of dysplasia 2) increased myeloblasts, but less that 20% of blood of marrow 3) presence of a clonal cytogenetic abnormality

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8
Q

What percentage of the cells in one lineage (erythroid, granulocytes, megakaryocytes) need appear dysplastic to qualify as dyshematopoiesis

A

10%

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9
Q

What are some morphologic features to look for dyserythropoiesis (3)

A

1) Megaloblastoid chromatin patterns in RBC Precursors, 2) Nuclear Irregularities (shape, number, ect.), 3) Prominent ring sideroblasts

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10
Q

What are some morphologic features to look for dysgranulopoiesis (3)

A

1) hypogranular, 2) megaloblastoid chromatin, 3) Pelgeroid neutrophils (bilobed nuclei)

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11
Q

What are some morphologic features to look for dysmegakaryopoiesis (2)

A

1) small, 2) hypolobated or non-lobated nuclei

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12
Q

list some common MDS-related abnormalites (4)

A

1) deletion, whole or partial, of chr 5 and or 7, 2) isolated deletion 5q, 3) trisomy 8 ? and 4) others

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13
Q

Define low grade MDS

A

myeloblsts account for <2% of blood cells

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14
Q

Define high grade MDS

A

Myeloblasts account for 5-19% of marrow cells and/or 3-19% of blood cells

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15
Q

T or F: Refractory cytopenia with unilinage dysplasia (RCUD) is a low grade MDS with dysplasia in only one lineage

A

TRUE

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16
Q

T or F: RCUD mostly has cases of refractory anemia and has a good prognosis

A

TRUE, median survival is >5 yrs and only 2% of cases transform to AML by year 5

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17
Q

T or F: Refractory cytopenia with multilineage dysplasia (RCMD) is a low grade MDS with dysplasia in only one lineage

A

TRUE

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18
Q

T or F: RCMD has a better prognosis than RCUD

A

FALSE, median survival is 2.5 yrs and 10% of cases turn to AML

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19
Q

What are the 2 types of high grade MDS?

A

Refractory Anemia with Excess Blasts-1 (RAEB-1) and Refractory Anemia with Excess Blasts-2 (RAEB-2)

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20
Q

Give some features of RAEB-1

A

5-9% blasts in marrow, and/or 2-4% blasts in blood, dismal prognosis, median 16 mon survival, 25% of cases will transform to AML

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21
Q

Give some features of RAEB-2

A

10-19% blasts in marrow, and/or 5-19% blasts in blood, very dismal prognosis, median 9 mon survival, 33% of cases will transform to AML

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22
Q

list 4 secondary myelodysplasias that can mimic MDS

A

1) vitamin deficiency (B12, folate, etc), 2) Toxin exposure (heavy metals), 3) exposure to certain drugs 4) viral infections

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23
Q

Define Myeloproliferative neoplasms

A

MPN, clonal hematopoietic neoplasm arising from hematopoietic stem cell.

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24
Q

T or F: MPN is seen in children most often

A

FALSE

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25
Q

T or F: MPN has clones replacing normal marrow cells in multiple lineages and gives rise to increased numbers of normal blood cells in one or more linages

A

TRUE, and blood cells are usually not dysplastic

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26
Q

what are some common themes in MPN (4)

A

1) increased # in blood cell type/s 2) increase in marrow cellularity 3) splenomegaly and/or hepatomegaly 4) insidious onset

27
Q

If MPN is untreated, what doe it progress to?

A

excessive marrow fibrosis with resultant bone marrow failue and transformation to acute leukemia

28
Q

T or F: MPN transformation to acute leukemia is higher than it is for MDS

A

FALSE

29
Q

What is chronic myelogenous leukemia (CML)?

A

MPN, manifests primarily as persistent neutrophilic leukocytosis, presence of BCR-ABL1 gene fusion

30
Q

clinical findings in CML

A

non-specific signs/symptoms like night sweats, weight loss, splenomegaly, and anemia. Minority are asymptomatic

31
Q

What is the typical age of diagnosis for CML?

A

40-60

32
Q

T or F: initial phase of CML is the chronic phase and it is when the majority of diagnoses are made

A

TRUE

33
Q

Chronic phase of CML is characterized by what?

A

usually stable, prominent leukocytosis due to neutrophilia, increased basophils, increased platlets, hypercellular bone marrow with prominent granulocytic hyperplasia

34
Q

What is the blast phase of CML and how does it come about?

A

If untreated, you get 20% or more blasts in the marrow or blood

35
Q

What is more common in CML-blast phase, more myeloblasts or lymphoblasts?

A

myeloblasts

36
Q

T or F: CML can go directly from chronic phase to blast phase, or go through an intermediate accelerated phase

A

TRUE

37
Q

What is the Philadelphia chromosome

A

BCR-ABL fusion gene. Translocation t(9;22)

38
Q

Whats the difference between BCR-ABL in CML cs Ph+ ALL

A

presence of p210 fusion protein, though some p190 may be found

39
Q

What do we treat CML with

A

protein tyrosine kinase inhibitors (PTLIs) such as GLEEVAC

40
Q

Why come we need 2nd and 3rd gen PTKIs?

A

some patients with CML develop resistance to imatinib (gleevac) through mutations

41
Q

What is polycythemia vera (PV)

A

MPN characterized by an increase in RBC mass

42
Q

What other findings can apear in PV

A

increased neutrophils and platelets in blood, trilineage hyperplasia in marrow, and bizarre megakaryocytes in marrow

43
Q

What do most all cases of PV have cytogenetically?

A

activating mutation of JAK2, usually V617F mutation

44
Q

If a patient has persistent erythocytosis w/o demonstrable JAK2 mutation, what secondary erythrocytosis causes should you expect?

A

smokers (due to carboxyhemoglobin), chronic hypoxia, certain hemoglobin disorders

45
Q

when is PV usually dianosed?

A

polycythemic phase with increased blood cell counts

46
Q

What does PV progress to

A

spent phase (aka post-PV myelofibrosis), with extensive marrow fibrosis and a fall in blood cell count

47
Q

What are clinical presenting symptoms of PV

A

headaches, dizziness, plethora (dusky, reddish skin), pruritus (itchy), paresthesia (pin and needle sensation), splenomegaly (70%), hepatomegaly (40%)

48
Q

What are the most serious complications of PV

A

venous and arterial thrombosis leading to DVT, MI, or stroke

49
Q

Thrombosis of what should raise suspicion of PV

A

mesenteric vein, portal vein, or splenic vein

50
Q

T or F: PV has a good prognosis

A

TRUE, survival times are 10-20 years

51
Q

What are the main therapeutics of PV

A

serial phlebotomy and aspirin therapy

52
Q

What do you give for problematic symptoms of PV and what can it cause?

A

mild chemotherapy, but this increases risk of transformation to acute leukemia

53
Q

What is Primary myelofibrosis

A

PMF is an MPN characterized by granulocytic and megakaryocytic hyperplasia, but no erythrocytosis.

54
Q

T or F: JAK2 mutations are never seen in PMF

A

FALSE, seen in 50% of cases

55
Q

What stage does PMF start in?

A

prefibrotic stage, which has increased platelets, increased neutrophils, hypercellular marrow, and granulocytic and megakaryocytic hyperplasia

56
Q

What does the fibrotic stage of PMF see

A

reticulin fibrosis in marrow, leukoerythroblastosis in blood, falling blood cell counts, extramedullary hematopoiesis leading to organomegaly

57
Q

what histologic features are seen in PMF fibrotic stage

A

immature granulocyts and red cells in blood, darcocytes (tear drops RBC), streaming appearance of marrow and bizarre entrapped megakaryocytes

58
Q

what is prognosis of PMF

A

not great. Most deaths due to bone marrow failure, minority become AML, survival if diagnosed in fibotic stage is 5 years

59
Q

what is Essential thrombocythemia

A

ET is an MPN characterized by persistent thrombocytosis, lacks marrow granulocytic hyperplasia, atypical megakaryocytes in ET are even larger than PMF.

60
Q

T or F: JAK2 mutations are present in 50% of ET cases

A

TRUE

61
Q

If present, what are some signs and symptoms of ET

A

transient ischemic attacks (TIAs), digital ischemia, arterial and venous thrombosis (less common than PV)

62
Q

T or F: Splenomegaly is common in Et

A

FALSE

63
Q

What is the prognosis of ET?

A

good, indolent disease with survivals of over 10 years, and transformation to myelofibrosis or AML is rare

64
Q

T or F: if asymptomatic, ET is diagnosed with CBC results

A

TRUE

Blood and lymph Unit II (6 decks)

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