Hubbard - MDS, MPS, Plasma Cell Disorders Flashcards
Sx’s = pancytopenia w/ hyperplastic marrow d/t clonal abnormalities of BM cells w/ varying degrees of cytopenias affecting one or more cell lines
MDS
What are the LDH and Ferritin levels in LDH
Elevated
What is the Tx for MDS 5q-
Lenalidomide
What is Chronic Myelomonocytic Leukemia (CMML)
aSx MDS w/ monocytosis
What mutations are seen in MDS
Chrom 5, 7, 9 deletions
Inv(16)
Trisomy 8
SF3B1
MDS is associated with exposure to benzene, radiation, chemo, and …
Petrochemicals (oil, agriculture)
MDS can transform to…
AML
What is the Tx for MDS
aSx Hx = supportive
Sx Hx = PRBCs
What factors indicate a poor prognosis in MDS
Monosomy 7
Hypodiploidy
Multiple mutations
What is RARS
Refractory Anemia w/ Ringed Sideroblasts
How do you make the Dx of RARS
Give pt Pyridoxine (B6) and repeat test in 6 months:
If no change, pt has RARS
What is the Tx for Polycythemia Vera
Phlebotomy
What can Polycythemia Vera progress to
Myelofibrosis
Acute Leukemia
What is the triad of Sx’s for Idiopathic/1˚ Myelofibrosis
Leukoerythroblastic anemia
Poikilocytosis
Splenomegaly
What mutations are seen in Idiopathic/1 Myelofibrosis
JAK2
CALR
What is the Tx for Idiopathic/1˚ Myelofibrosis
aSx Hx = supportive
Sx Hx = Ruxolitinib (JAK2 inhibiotor)
What mutations are seen in Essential Thrombocytosis
JAK2
CALR
What is the criteria for Dx of MM
BM plasmacytosis > 10%
Lytic bone lesins
Elevated M-Protein
What is the Tx for MGUS
Observation
What is the Tx for MM
Bisphosphanates
Thalidomide, Bortezomib, Dexamethasone
What is the Tx for Smoldering Myeloma
Observation
What is the Tx for Solitary Myeloma
Radiation
Sx’s = progressive sensorimotor polyneuropathy w/ sclerotic bone lesions, and hyperpigmentation
POEMS Syndrome
What male and female specific Sx’s may be seen in POEMS Syndrome
Male = impotence/gynecomastia Female = amenorrhea
Which HCD affects Waldeyer’s ring
g-HCD
What is the Tx for g-HCD
Rituximab
Which HCD has chronic diarrhea
a-HCD
Which HCD is associated with CLL
µ-HCD
Sx’s = BM involvement w/ no bone lesions or hypercalcemia but large M spike
Waldenstrom’s Macroglobulinemia
Lymphoplasmacytic Lymphoma
Sx’s = epistaxis, HSM, neuro Sx’, “sausage-like” retinal veins
Waldenstrom’s Macroglobulinemia
What is the Tx for Waldenstrom’s Macroglobulinemia
Plasmapheresis
Sx’s = clonal B cell disorder that stains + in Congo red
AL Amyloidosis
What is the Tx for Amyloidosis
IV Melphalan
