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Heme and Lymph Final > DSA: Myelodysplasia and Plasma Disorders > Flashcards

DSA: Myelodysplasia and Plasma Disorders Flashcards

1
Q

What are melody spastic syndromes?

A
  • disorders of the pluripotential stem cell

- chapter idea by ineffective hematopoietic

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2
Q

Clinical picture of MDS

A
  • pancytopenia with hyperplastic marrow

- potential risk for development of a cut leukemia

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3
Q

What’s a big etiology for MDS?

A

Chemotherapy…. We damage the DNA

-big offender is the alkylating agents

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4
Q

Alkylating agents

A
  • cyclophosphamide
  • ifosfamide
  • cisplatin
  • caroplatin
  • nitrogen mustard
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5
Q

Anthracyclin Abx

A

The rubicins

-also cause MDS

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6
Q

Radiation as an etiology of MDS

A

Atomic bomb blast survivors

  • nuclear accidents
  • therapeutic radiation…. Lymphoma, others
  • petrochemical exposure
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7
Q

Demographics of MDS

A

-mostly elderly ppl…. 6 and 7 decade

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8
Q

Cytogenetics of MDS

A
  • partial or total loss. Of chromosome 5 or 7
  • inv 16 (not the same as AML)
  • trisomy 8
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9
Q

Constitutional symptoms of MDS

A
  • a symptomatic in half of them
  • fatigue
  • pallor
  • bleeding
  • infection
  • pancytopenia
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10
Q

Laboratory abnormalities in MDS

A
  • elevated serum LDH

- iron overload… Increased serum ferritin… TIBC will be normal

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11
Q

What could we suggest as a diagnosis in a patient with pancytopenia

A
  • hypersplenism
  • aplastic anemia
  • myeoldysplasia
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12
Q

Refractory anemia with ringed sideroblasts (RARS)

A
  • like RA
  • ringed sideroblasts in marrow precursors
  • lowest risk of conversion of AML (10-15%)
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13
Q

What are the ringed sideroblasts

A

Mitochondria laden with Fe encircling the nucleus of the erythropoietin precursors
-no clear explanation for cause

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14
Q

Pyridoxine deficiency (vit B6)

A
  • some puts with anemia are found to have ringed sideroblasts on marrow exam secondary to B6 deficiency
  • so check B6 level on every patient with ringed sideroblasts
  • if they get better with B6 replacement, if it doesn’t work, it’s ARAS and that’s got a bad prognosis
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15
Q

Prognosis of myeoldysplasias in general

A

-poor

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16
Q

Adverse prognostic features

A
  • marrow blasts >5%
  • platelets <100,000
  • Hbg < 10 g
  • Neutrophils <2500
  • Age>60 years
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17
Q

Cytogenetic abnormalities with a poor prognosis

A
  • monopsony 7
  • hypodiploidy
  • multiple abnormalities
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18
Q

Favorable prognosis with cytogenetic abnormalities

A

5q syndrome- beneficial responses to lenalidomide reported

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19
Q

Tx of myelodysplasia

A
  • supportive care
  • avoid meds that damage marrow
  • aggressive tx of infections
  • transfuse PRBC’s when symptomatic
  • transfuse platelets only for bleeding or in prep for surgery
  • watch for iron overload-desferrioxamine or deferasirox if present
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20
Q

Supportive care with hematinics

A
  • sump vitamins not needed if chemical assays normal

- B6 maybe but you have to do it for 6 months

21
Q

Supportive care with EPO

A
  • expensive

- serum EPO level >500 indicates poor response

22
Q

What kinds of therapy do we use for myelodysplasia

A

-hypo methylation agents: azacitidine, decitabine… Direct cytotoxicity on abnormal bone marrow

23
Q

High intensity therapy

A
  • AML induction-style treatment
  • not as effective as denomination AML
  • these patients do not do as well
  • stem cell transplantation is not as good
24
Q

Myelofibrosis definition

A

Primary myeloproliferative disorder characterized by marrow fibrosis and extra medullary hematopoiesis
-differentiate from secondary myelofibrosis from other myeloproliferative disorders or malignancy

25
Q

Triad with myelofibrosis

A

Leukoerythroblastic anemia
Poikilocytosis
Splenomegaly

26
Q

Pathogenesis of myelofibrosis

A
  • increased reticulum deposition in marrow
  • maybe secondary to increased PDGF and other cytokines in marrow
  • marrow architecture disrupted with subsequent mobilization of marrow stem cells to extra medullary sites
27
Q

What kinds of mutations are in myelofibrosis

A

JAK2 mutations

-45-65%

28
Q

Disease course for myelofibrosis

A

-progressive pancytopenia and organometallic indicate development of later stages of disease

29
Q

Tx of myelofibrosis

A
  • manage anemia with transfusions
  • treat infections aggressively (those are the major cause of death)
  • hydroxyurea for Splenomegaly….
30
Q

What is ruxolitinib

A

-new JAK inhibitor approved for tx of intermediate and high risk myelofibrosis

31
Q

Is there a standardized tx for myelofibrosis?

A

No

-asymptomatic patients can be observed

32
Q

What is the only curative treatment for myelofibrosis

A

Allogenic stem cell transplantation if they can do it

33
Q

Definition of plasma cell dyscrasia

A

-a condition in which there is colonial proliferation and immortalization of Ig-secreting plasma cells

34
Q

When do most plasma cell disorder occur?

A

Middle aged and older ppl

35
Q

What is more common in the malignant plasma cell disorders?

A

Presenting complaints of fatigue, weight loss, bone pain, infection, etc…
-less common (peripheral neuropathy) can occur with any plasma cell dyscrasia

36
Q

Can you do OMM on a patient with a suspected plasma cell disorder?

A

Yeah but be super careful… Their bones are super brittle

37
Q

What is hyperviscosity most common with?

A

Macroglobulinemia, it’s rare with the other plasma cell disorders

38
Q

If a patient has an albumin globulin ratio of <1, what should we suggest as a potential diagnosis?

A

A plasma cell disorder

39
Q

What do we do with a person who has a solitary plasma cytoma?

A

Give them radiation to that solitary site

-no need for chemo, don’t make all of them sick

40
Q

What is the normal value for Calcium

A

8.5-10.5

41
Q

What does elevated calcium have an effect on

A

The kidney a lot, it is like a diuretic so pts will come in and be pretty dehydrated

42
Q

In myeloma, what happens to the normal plasma cells?

A

They get shut down!

  • that is why there is just the gamma spike without any kind of “shoulder”
  • helps us sort out multiple myeloma from MGUS
43
Q

If we do a bone scan in multiple myeloma, what will we see?

A

Nothing, because there are no blasts to take it up, there are only clasts going to work
-DONT DO A BONE SCAN WITH MYELOMA, DO AN XRAY

44
Q

If cancer is the answer, what is the issue?

A

Tissue!!!! We need that for diagnosis

45
Q

What is presbyccusis?

A

Hearing loss of old age

46
Q

Waldenstrom gammapothy

A

Has hyperviscosity

-they eye looks like sausage links

47
Q

What else can cause hyperviscosity and sausage linking?

A

Polycythemia Vera

48
Q

What is roleaux formation?

A

When the blood cells stick together like poker chips

-can happen in Multiple myeloma or waldenstrom’s

49
Q

How do you manage waldenstrom?

A
  • plasmapheresis

- treatment of underlying disease

Heme and Lymph Final (14 decks)

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