276b BM failure

Question 1

Aplastic anemia - what is it? what can cause it?

Answer 1

failure/destruction of myeloid stem cells (hematopoietic precursors)

due to:
most are idiopathic (autoimmune?)
radiation/drugs
viruses (B19, EBV, HIV, HCV)
Fanconi anemia --> defective DNA repair/telemere length

Question 2

Aplastic anemia - what do cells look like on smear? BM biopsy?

Answer 2

normal cell morphology, but severe pancytopenia (anemia, leukopenia, thrombocytopenia)

BM is hypocellular w/ more fat (takes up entire space)

Question 3

Aplastic anemia - symptoms?

Answer 3

fatigue, malaise, pallor

purpura, mucosal bleeding

infection

Question 4

Aplastic anemia - Rx?

Answer 4

immunosuppression

BM transplant (allogeneic)

transfusion

CSF

Question 5

Myelodysplastic syndrome - what is it?

Answer 5

stem cell disorders (clonal, malignant HSC) –> ineffective hematopoiesis –> defective cell maturation of all non-lymphoid lineages

Question 6

Myelodysplastic syndrome - risk of what disease?

Answer 6

AML (acute myelogenous leukemia)

Question 7

Myelodysplastic syndrome - typical PMN appearance? when do you see it? RBC?

Answer 7

Pseudo-Pelger-Huet anomaly

PMN with bilobed nuclei connected by a thin filament of chromatin

seen after chemo

ringed sideroblasts (Prussian blue –> stains Fe in ribosomes)

Question 8

Myelodysplastic syndrome - what are the findings?

Answer 8

low reticulocytes despite anemia, hypercellular BM

Question 9

Myelodysplastic syndrome - what causes it and what age group?

Answer 9

de novo mutations or environmental exposure (radiation, benzene, chemo)

> 65 years old

Question 10

Acute Myelogenous Leukemia (AML) - what is it?

Answer 10

clonal, malignant proliferation of myeloid stem cells that can’t differentiate into mature cells

Question 11

Acute Myelogenous Leukemia (AML) - who gets it?

Answer 11

65+ years

Question 12

Acute Myelogenous Leukemia (AML) - risk factors?

Answer 12

chemo (alkylating), radiation, myeloproliferative disorders, DS

Question 13

Acute Myelogenous Leukemia (AML) - findings

Answer 13

Auer rods

Peroxidase + cytoplasmic inclusions

increased circulating myeloblasts on smear

Question 14

Acute Myelogenous Leukemia (AML) - clinical features?

Answer 14

fatigue, fever, sweats

infections

cytopenias (anemia, thrombocytopenia)

leukocytosis

coagulation disorders –> DIC

Question 15

Acute Lymphoblastic Leukemia/lymphoma (ALL) - who gets it?

Answer 15

<15 years

kids with DS

Question 16

Acute Lymphoblastic Leukemia/lymphoma (ALL) - presentation

Answer 16

mediastinal mass - big thymus –> superior vena cava syndrome

Question 17

Acute Lymphoblastic Leukemia/lymphoma (ALL) - BM and smear? cell markers?

Answer 17

increased lymphoblasts

TdT+ (marker of pre-T and pre-B cells)
CD10, 20+ (pre B cells only)
CD3 (T cell marker)

Question 18

Acute Lymphoblastic Leukemia/lymphoma (ALL) - clinical features?

Answer 18

CNS and testes can be involved

BM failure - pallor, lethargy, infection

weight loss

unintentional weight loss

Question 19

Acute Lymphoblastic Leukemia/lymphoma (ALL) - morphology

Answer 19

lymphoblasts - have huge nuclei and sometimes vacuoles

Question 20

Acute Lymphoblastic Leukemia/lymphoma (ALL) - cytogenetics with good prognosis

Answer 20

t(12:21)