DSA: Myelodysplasia and Plasma Disorders

Question 1

What are melody spastic syndromes?

Answer 1

• disorders of the pluripotential stem cell

- chapter idea by ineffective hematopoietic

Question 2

Clinical picture of MDS

Answer 2

• pancytopenia with hyperplastic marrow

- potential risk for development of a cut leukemia

Question 3

What’s a big etiology for MDS?

Answer 3

Chemotherapy…. We damage the DNA

-big offender is the alkylating agents

Question 4

Alkylating agents

Answer 4

• cyclophosphamide
• ifosfamide
• cisplatin
• caroplatin
• nitrogen mustard

Question 5

Anthracyclin Abx

Answer 5

The rubicins

-also cause MDS

Question 6

Radiation as an etiology of MDS

Answer 6

Atomic bomb blast survivors

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

Question 7

Demographics of MDS

Answer 7

-mostly elderly ppl…. 6 and 7 decade

Question 8

Cytogenetics of MDS

Answer 8

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

Question 9

Constitutional symptoms of MDS

Answer 9

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

Question 10

Laboratory abnormalities in MDS

Answer 10

• elevated serum LDH

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

Question 11

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

Answer 11

• hypersplenism
• aplastic anemia
• myeoldysplasia

Question 12

Refractory anemia with ringed sideroblasts (RARS)

Answer 12

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

Question 13

What are the ringed sideroblasts

Answer 13

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

Question 14

Pyridoxine deficiency (vit B6)

Answer 14

• 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

Question 15

Prognosis of myeoldysplasias in general

Answer 15

-poor

Question 16

Adverse prognostic features

Answer 16

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

Question 17

Cytogenetic abnormalities with a poor prognosis

Answer 17

• monopsony 7
• hypodiploidy
• multiple abnormalities

Question 18

Favorable prognosis with cytogenetic abnormalities

Answer 18

5q syndrome- beneficial responses to lenalidomide reported

Question 19

Tx of myelodysplasia

Answer 19

• 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

Question 20

Supportive care with hematinics

Answer 20

• sump vitamins not needed if chemical assays normal

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

Question 21

Supportive care with EPO

Answer 21

• expensive

- serum EPO level >500 indicates poor response

Question 22

What kinds of therapy do we use for myelodysplasia

Answer 22

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

Question 23

High intensity therapy

Answer 23

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

Question 24

Myelofibrosis definition

Answer 24

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

Question 25

Triad with myelofibrosis

Answer 25

Leukoerythroblastic anemia Poikilocytosis Splenomegaly

Question 26

Pathogenesis of myelofibrosis

Answer 26

- 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

Question 27

What kinds of mutations are in myelofibrosis

Answer 27

JAK2 mutations | -45-65%

Question 28

Disease course for myelofibrosis

Answer 28

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

Question 29

Tx of myelofibrosis

Answer 29

- manage anemia with transfusions - treat infections aggressively (those are the major cause of death) - hydroxyurea for Splenomegaly....

Question 30

What is ruxolitinib

Answer 30

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

Question 31

Is there a standardized tx for myelofibrosis?

Answer 31

No | -asymptomatic patients can be observed

Question 32

What is the only curative treatment for myelofibrosis

Answer 32

Allogenic stem cell transplantation if they can do it

Question 33

Definition of plasma cell dyscrasia

Answer 33

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

Question 34

When do most plasma cell disorder occur?

Answer 34

Middle aged and older ppl

Question 35

What is more common in the malignant plasma cell disorders?

Answer 35

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

Question 36

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

Answer 36

Yeah but be super careful... Their bones are super brittle

Question 37

What is hyperviscosity most common with?

Answer 37

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

Question 38

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

Answer 38

A plasma cell disorder

Question 39

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

Answer 39

Give them radiation to that solitary site | -no need for chemo, don't make all of them sick

Question 40

What is the normal value for Calcium

Answer 40

8.5-10.5

Question 41

What does elevated calcium have an effect on

Answer 41

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

Question 42

In myeloma, what happens to the normal plasma cells?

Answer 42

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

Question 43

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

Answer 43

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*

Question 44

If cancer is the answer, what is the issue?

Answer 44

Tissue!!!! We need that for diagnosis

Question 45

What is presbyccusis?

Answer 45

Hearing loss of old age

Question 46

Waldenstrom gammapothy

Answer 46

Has hyperviscosity | -they eye looks like sausage links

Question 47

What else can cause hyperviscosity and sausage linking?

Answer 47

Polycythemia Vera

Question 48

What is roleaux formation?

Answer 48

When the blood cells stick together like poker chips | -can happen in Multiple myeloma or waldenstrom's

Question 49

How do you manage waldenstrom?

Answer 49

- plasmapheresis | - treatment of underlying disease