Pancytopenia and Bone Marrow Failure Syndromes Flashcards Preview

MDCN 350: Course 1 > Pancytopenia and Bone Marrow Failure Syndromes > Flashcards

Flashcards in Pancytopenia and Bone Marrow Failure Syndromes Deck (16)
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1
Q

does she have pancytopenia?

A

yes. deficient in more than one cell line: low RBC, and low WBC (neutrophils super low but still organised in NLMEB)

2
Q

clinical presentations of pancyotpenia

A

very broad

anemia symptoms: easy bruising, fatigue, heavy periods (cause of anemia)

platelets issues: easy bruising, mucosal bleeding, longer bleeding

WBC issues: sinopulmonary infections

3
Q

3 broad reasons for pancytopenia

A

decreased production

incresed destruction

sequestration

4
Q

why might pancytopenia arise from a production problem?

A
  1. nutritional deficiency (B12/folate)
  2. infiltrative marrow – hematological malignancy or nonhematological maligancy, or myelofibrosis
  3. stem cell damage – chemo, toxins, aplastic anemia, myelodysplasia
5
Q

causes of normocytic anemia

A

anemia of chornic inflammatory disease

  • early iron def

- bone marrow aplasia, suppression of infiltraiton

  • renal failure
  • myelodysplasia

- hemolysis or acute blood loss

-sequestration.

6
Q

what test best definitively diagnoses pancytopenia?

A

bone marrow biopsy

then do microscopy, stains for immunohistochemistry, flow cytometry, karyotype, and DNA analysis

7
Q

if a patient had aplastic anemia resulting in anemia and panctopenia, would retic count be high to compensate?

A

no, it would be low because there are no proliferative cells in the bone marrow to begin with.

8
Q

outline inherited and acquired causes of aplastic anemia

A

inherited: fanconis anemia, dyskaratosis congenita.

acquired; radiation and chemo, toxins (benzene), infection like HIB, EBV, parvovirus

  • drug reactions like NSAIDs, Antithyroids and antiepileptics etc.
  • immune disorders
  • thymoma
  • pregnancy.
9
Q

aplastic anemias are largely correlated by ___ ___ disorders

A

autoimmune disorders. if a patient actually has an autoimmune disorder, they can recover cellularity if they receive immune suppression (ex/ methotrexate, predisone, retuximab, cyclosporin)

10
Q

general treatment of aplastic anemia or bone marrow failure resulting in pancytopenia

A

plus immune suppression or hematopoietic cell transplantation.

immune suppression via ATG (antithrymocyte globulin), steroids, cyclosporine, sometimes growth factors like GSCF

11
Q

how do thrombopoietin receptor agonists help Aplastic anemia

A
  • thrombopoietin receptors are expressed by primitive hematopoietic stem cells
  • thrombopoietin potentiates stem cell expansion
  • higher repsonse rates but uncertain risk of pormoting leukemia when added to immune suppression.
12
Q

risks of hematopoietic cell transplantation for SAA

A
  • the only curative therapy but toxicities can be limiting
  • risk of GvHD– need cyclosporin or steroids or methotrexate or something
  • risk of infertility.
13
Q

Case: a 38 yo woman is short of breath and tired. She had a normal delivery of a healthy baby girl 4 months prior. She is finding it more difficult to run long distances. She experiences heavy bruising. PBS shows this:

A

auer rods present. huge cell is a blast. this is AML.

14
Q

a 58yo woman presents to ER sent in by walk-in clinic with abnomrla blood work after presenting wiht fatigue.

Hb 45, MCV 120, WBC 1.2 with neutrophils 0.8, platelets 60.

interpret her PBS and labs

A

Hb= very anemic.

Macrocytic anemia

WbC= low, but neutrophils probably are still wihtin proportion.

she’s pancytopenic for sure. platelets also low.

everything is affected- Aplastic anemia? What accounts for MCV? nutritional deficiency?

also, PBS shows hypersegmentation in neutrophil– characteristic of B12 deficiency

CASE ANSWER: B12 folate was low. showcases hematopoietic ineffectiveness.

15
Q

Case: a patient with longstanding prostate cancer is noted to have progressively increasing PSA.

  • there is bone pain, on narcotics

CBC: Hb92, MCV 85, Platelets 32, neutrophils 1, with 0.1 myelocytes and 0.1 metamyelocytes seen

  • nucleated red blooc cells and tear drop cells seen on PBS.

interpret results and outline Dx/what you’d you next

A

he is anemia, normocytiv anemia (chronic disease? bleeding?) low platelets, low neutorphils– pancytopenic.

  • nucleated reds blood cells and tear drop cells indicate a big malignancy that is causing them to be squeezed out prematurely.
  • I’d do a BM aspirate- check for infiltration/metastasis from prostate cancer?
  • bone marrow aspirate shows marrow infiltrated with carcinoma cells.
16
Q

summary:

A