DSA: Myelodysplasia and Plasma Disorders Flashcards
What are melody spastic syndromes?
- disorders of the pluripotential stem cell
- chapter idea by ineffective hematopoietic
Clinical picture of MDS
- pancytopenia with hyperplastic marrow
- potential risk for development of a cut leukemia
What’s a big etiology for MDS?
Chemotherapy…. We damage the DNA
-big offender is the alkylating agents
Alkylating agents
- cyclophosphamide
- ifosfamide
- cisplatin
- caroplatin
- nitrogen mustard
Anthracyclin Abx
The rubicins
-also cause MDS
Radiation as an etiology of MDS
Atomic bomb blast survivors
- nuclear accidents
- therapeutic radiation…. Lymphoma, others
- petrochemical exposure
Demographics of MDS
-mostly elderly ppl…. 6 and 7 decade
Cytogenetics of MDS
- partial or total loss. Of chromosome 5 or 7
- inv 16 (not the same as AML)
- trisomy 8
Constitutional symptoms of MDS
- a symptomatic in half of them
- fatigue
- pallor
- bleeding
- infection
- pancytopenia
Laboratory abnormalities in MDS
- elevated serum LDH
- iron overload… Increased serum ferritin… TIBC will be normal
What could we suggest as a diagnosis in a patient with pancytopenia
- hypersplenism
- aplastic anemia
- myeoldysplasia
Refractory anemia with ringed sideroblasts (RARS)
- like RA
- ringed sideroblasts in marrow precursors
- lowest risk of conversion of AML (10-15%)
What are the ringed sideroblasts
Mitochondria laden with Fe encircling the nucleus of the erythropoietin precursors
-no clear explanation for cause
Pyridoxine deficiency (vit B6)
- 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
Prognosis of myeoldysplasias in general
-poor
Adverse prognostic features
- marrow blasts >5%
- platelets <100,000
- Hbg < 10 g
- Neutrophils <2500
- Age>60 years
Cytogenetic abnormalities with a poor prognosis
- monopsony 7
- hypodiploidy
- multiple abnormalities
Favorable prognosis with cytogenetic abnormalities
5q syndrome- beneficial responses to lenalidomide reported
Tx of myelodysplasia
- 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
Supportive care with hematinics
- sump vitamins not needed if chemical assays normal
- B6 maybe but you have to do it for 6 months
Supportive care with EPO
- expensive
- serum EPO level >500 indicates poor response
What kinds of therapy do we use for myelodysplasia
-hypo methylation agents: azacitidine, decitabine… Direct cytotoxicity on abnormal bone marrow
High intensity therapy
- AML induction-style treatment
- not as effective as denomination AML
- these patients do not do as well
- stem cell transplantation is not as good
Myelofibrosis definition
Primary myeloproliferative disorder characterized by marrow fibrosis and extra medullary hematopoiesis
-differentiate from secondary myelofibrosis from other myeloproliferative disorders or malignancy
Triad with myelofibrosis
Leukoerythroblastic anemia
Poikilocytosis
Splenomegaly
Pathogenesis of myelofibrosis
- 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
What kinds of mutations are in myelofibrosis
JAK2 mutations
-45-65%
Disease course for myelofibrosis
-progressive pancytopenia and organometallic indicate development of later stages of disease
Tx of myelofibrosis
- manage anemia with transfusions
- treat infections aggressively (those are the major cause of death)
- hydroxyurea for Splenomegaly….
What is ruxolitinib
-new JAK inhibitor approved for tx of intermediate and high risk myelofibrosis
Is there a standardized tx for myelofibrosis?
No
-asymptomatic patients can be observed
What is the only curative treatment for myelofibrosis
Allogenic stem cell transplantation if they can do it
Definition of plasma cell dyscrasia
-a condition in which there is colonial proliferation and immortalization of Ig-secreting plasma cells
When do most plasma cell disorder occur?
Middle aged and older ppl
What is more common in the malignant plasma cell disorders?
Presenting complaints of fatigue, weight loss, bone pain, infection, etc…
-less common (peripheral neuropathy) can occur with any plasma cell dyscrasia
Can you do OMM on a patient with a suspected plasma cell disorder?
Yeah but be super careful… Their bones are super brittle
What is hyperviscosity most common with?
Macroglobulinemia, it’s rare with the other plasma cell disorders
If a patient has an albumin globulin ratio of <1, what should we suggest as a potential diagnosis?
A plasma cell disorder
What do we do with a person who has a solitary plasma cytoma?
Give them radiation to that solitary site
-no need for chemo, don’t make all of them sick
What is the normal value for Calcium
8.5-10.5
What does elevated calcium have an effect on
The kidney a lot, it is like a diuretic so pts will come in and be pretty dehydrated
In myeloma, what happens to the normal plasma cells?
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
If we do a bone scan in multiple myeloma, what will we see?
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
If cancer is the answer, what is the issue?
Tissue!!!! We need that for diagnosis
What is presbyccusis?
Hearing loss of old age
Waldenstrom gammapothy
Has hyperviscosity
-they eye looks like sausage links
What else can cause hyperviscosity and sausage linking?
Polycythemia Vera
What is roleaux formation?
When the blood cells stick together like poker chips
-can happen in Multiple myeloma or waldenstrom’s
How do you manage waldenstrom?
- plasmapheresis
- treatment of underlying disease
