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Zak, Heme/Lymph > Hubbard - MDS, MPS, Plasma Cell Disorders > Flashcards

Hubbard - MDS, MPS, Plasma Cell Disorders Flashcards

1
Q

Sx’s = pancytopenia w/ hyperplastic marrow d/t clonal abnormalities of BM cells w/ varying degrees of cytopenias affecting one or more cell lines

A

MDS

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2
Q

What are the LDH and Ferritin levels in LDH

A

Elevated

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3
Q

What is the Tx for MDS 5q-

A

Lenalidomide

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4
Q

What is Chronic Myelomonocytic Leukemia (CMML)

A

aSx MDS w/ monocytosis

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5
Q

What mutations are seen in MDS

A

Chrom 5, 7, 9 deletions
Inv(16)
Trisomy 8
SF3B1

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6
Q

MDS is associated with exposure to benzene, radiation, chemo, and …

A

Petrochemicals (oil, agriculture)

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7
Q

MDS can transform to…

A

AML

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8
Q

What is the Tx for MDS

A

aSx Hx = supportive

Sx Hx = PRBCs

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9
Q

What factors indicate a poor prognosis in MDS

A

Monosomy 7
Hypodiploidy
Multiple mutations

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10
Q

What is RARS

A

Refractory Anemia w/ Ringed Sideroblasts

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11
Q

How do you make the Dx of RARS

A

Give pt Pyridoxine (B6) and repeat test in 6 months:

If no change, pt has RARS

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12
Q

What is the Tx for Polycythemia Vera

A

Phlebotomy

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13
Q

What can Polycythemia Vera progress to

A

Myelofibrosis

Acute Leukemia

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14
Q

What is the triad of Sx’s for Idiopathic/1˚ Myelofibrosis

A

Leukoerythroblastic anemia
Poikilocytosis
Splenomegaly

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15
Q

What mutations are seen in Idiopathic/1 Myelofibrosis

A

JAK2

CALR

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16
Q

What is the Tx for Idiopathic/1˚ Myelofibrosis

A

aSx Hx = supportive

Sx Hx = Ruxolitinib (JAK2 inhibiotor)

17
Q

What mutations are seen in Essential Thrombocytosis

A

JAK2

CALR

18
Q

What is the criteria for Dx of MM

A

BM plasmacytosis > 10%
Lytic bone lesins
Elevated M-Protein

19
Q

What is the Tx for MGUS

A

Observation

20
Q

What is the Tx for MM

A

Bisphosphanates

Thalidomide, Bortezomib, Dexamethasone

21
Q

What is the Tx for Smoldering Myeloma

A

Observation

22
Q

What is the Tx for Solitary Myeloma

A

Radiation

23
Q

Sx’s = progressive sensorimotor polyneuropathy w/ sclerotic bone lesions, and hyperpigmentation

A

POEMS Syndrome

24
Q

What male and female specific Sx’s may be seen in POEMS Syndrome

A 
Male = impotence/gynecomastia
Female = amenorrhea
25
Q

Which HCD affects Waldeyer’s ring

A

g-HCD

26
Q

What is the Tx for g-HCD

A

Rituximab

27
Q

Which HCD has chronic diarrhea

A

a-HCD

28
Q

Which HCD is associated with CLL

A

µ-HCD

29
Q

Sx’s = BM involvement w/ no bone lesions or hypercalcemia but large M spike

A

Waldenstrom’s Macroglobulinemia

Lymphoplasmacytic Lymphoma

30
Q

Sx’s = epistaxis, HSM, neuro Sx’, “sausage-like” retinal veins

A

Waldenstrom’s Macroglobulinemia

31
Q

What is the Tx for Waldenstrom’s Macroglobulinemia

A

Plasmapheresis

32
Q

Sx’s = clonal B cell disorder that stains + in Congo red

A

AL Amyloidosis

33
Q

What is the Tx for Amyloidosis

A

IV Melphalan

Zak, Heme/Lymph (15 decks)

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