Gp-Mksap-Heme-02

Front 1

Meaning of these on peripheral smear?

1. Microcytosis, anisocytosis

2. Spherocytes

3. Macrocytes or macroovalocytes

4. Target cells (codocytes)

5. Schistocytes

6. Nucleated erythrocytes

Back 1

1. Iron deficiency

2. Hereditary spherocytosis, warm autoimmune hemolytic anemia

3. Cobalamin or folate deficiency; myelodysplasia, use of antimetabolites

4. Hemoglobinopathy, liver disease, splenectomy

5. Microangiopathy (TTP, HUS, DIC)

6. Marrow stress (hemolysis, hypoxia)

Front 2

Meaning of these on peripheral smear?

1. Teardrop cells (dacryocytes)

2. Bite cells

3. Rouleaux

4. Burr cells (echinocytes)

5. Spur cells (acanthocytes)

Back 2

1. Fibrosis, marrow granuloma, marrow infiltration

2. G6PD deficiency

3. Paraproteinemia (myeloma)

4. Kidney disease

5. Severe liver disease

Front 3

=== ANEMIA ===

Back 3

=== ANEMIA ===

Front 4

Iron deficiency and Hepcidin?

Back 4

- Hepcidin causes decreased iron absorption from enterocytes and decreased iron release by macrophages through internalization and proteolysis of the membrane protein ferroportin.

- iron deficiency is characterized by low hepcidin levels

Front 5

i. Iron is absorbed predominantly in the

ii. how soon after starting iron can you check the results?

Back 5

i. proximal small bowelii. retic count will go up in 5 days

Front 6

test for folate deficiency?

Back 6

Serum homocysteine levels increase in folate deficiency, whereas BOTH homocysteine and methylmalonic acid levels are increased in cobalamin deficiency.

- Measuring serum folate levels is typically unreliable

Front 7

target hgb in anemia of CKD

Back 7

Current guidelines recommend the use of supplemental erythropoiesis-stimulating agents to achieve target hemoglobin values of 11 to 12 g/dL (110-120 g/L) in patients receiving dialysis.

Front 8

basic findings in hemolytic anemia

Back 8

An increased reticulocyte count,

increased indirect bilirubin

increased LDH levels,

decreased haptoglobin level

morphologic changes in erythrocytes

Front 9

Hemolytic anemia, in addition to correcting underlying condition, what to give pt?

Back 9

Folic acid 1 mg / day

Front 10

hereditary spherocytosis tx?

Back 10

asymptomatic: none

symptomatic: splenectomy

Front 11

alpha thal trait (-α/-α or --/αα), how to tell from iron deficiency?

Back 11

Presents similarly, but IDA has increased RDW, alpha thal has normal RDW

Front 12

Beta - thal trait findings

Back 12

mild anemia, microcytosis, hypochromia, target cells, and increased hemoglobin A2 (α2δ2) and, at times, hemoglobin F (α2γ2) on electrophoresis.

Front 13

Sickle trait (AS)

hgb findings, clinical features?

Back 13

NORMAL: Hgb, MCV, smear

Hb S < 50%, rest HbA

Can have splenic sequestration and hematuria

Crisis with severe hypoxia

Front 14

Sickle cell SC

electrophoresis finding, clinical features?

Back 14

Hgb 10-15, HbS 50% MCV 75-normal

Avascular necrosis

retinal infarcts

Front 15

Sickle cell HbSS

electrophoresis findings, clinical features?

Back 15

Hb 6-8, > 90% HbS

Vasocclusive crises, functional asplenia

Front 16

Warm autoimmune hemolytic anemia , dx?

Back 16

- The direct Coombs (antiglobulin) test is used

- typically positive for IgG and negative or only weakly positive for complement (C3) in warm autoimmune hemolytic anemia, whereas it is positive for only complement (C3) in cold agglutinin disease

Front 17

Warm autoimmune hemolytic anemia , tx?

Back 17

- steroids

- Splenectomy if non responsive

- refractory: azithioprine, cyclo(phos/sporin)

Front 18

Cold Agglutinin Disease, dx / tx?

Back 18

peripheral blood smear in CAD may show clumping, which leads to a falsely elevated MCV

- direct Coombs test is typically negative for IgG and strongly positive for complement

TX: cold avoidance, plasmapheresis if acute

Front 19

Microangiopathic Hemolytic Anemia, description?

Back 19

refers to the disruption, fragmentation, and subsequent lysis of erythrocytes during travel through the vascular system

Front 20

Microangiopathic Hemolytic Anemia, smear and lab findings?

Back 20

- Erythrocyte fragmentation leads to the smear finding of schistocytes or helmet cells

- Lab: intravascular hemolysis with low haptoglobin levels, hemoglobinuria, and elevated serum LDH

Front 21

Microangiopathic Hemolytic Anemia causes?

Back 21

Macrovascular: turbulen flow around mech valves, pumps, etc

Microvascular: TTP / HUS

Front 22

PNH, consider in pt with __?

Back 22

hemolytic anemia, pancytopenia, or unprovoked atypical thrombosis.

Front 23

PNH caused by? dx?

Back 23

- Mutations in the PIG-A gene -> absence of glycosylphosphatidylinositol

- absence of decay-accelerating factor (CD55) and membrane inhibitor of reactive lysis (CD59)

DX: flow cytometry with CD55 and CD59 deficiency

Front 24

PNH, tx?

Back 24

* Eculizumab (c5 compl Ab) has resulted in decreased transfusion requirements, improved quality of life, and potentially decreased thrombosis in transfusion-dependent patients with paroxysmal nocturnal hemoglobinuria.

Front 25

infectious causes of hemolysis?

Back 25

- Malaria (most common)

- Babesiosis: NE USA

- Clostridial sepsis (alpha toxin)

- Bartonellosis (s. america, sand fly)

Brown recluse spider, bee sting, snake bite

Front 26

tx for secondary iron overload?

Back 26

Deferasirox PO (can cause agranulo / AKI)

- deferoxamine IV

Front 27

==== TRANSFUSION ===

Back 27

==== TRANSFUSION ===

Front 28

FFP indications?

Back 28

- Multiple clotting deficiencies with active bleeding (e.g., DIC, liver disease)

- TTP

- Reversal of warfarin in patients with intracranial bleeding

- Factor replacement when specific factor concentrates not available

- Massive transfusion of packed red blood cells to avoid dilutional coagulopathy

Front 29

Indication for plt transfusion as prophylaxis?

Back 29

<10,000/µL in patients with leukemia with no other bleeding risk factors

<50,000/µL and planned surgery

<100,000/µL and planned intracranial surgery

Front 30

Indication for plt transfusion as treatment?

Back 30

Active bleeding with platelet count <50,000-100,000/µL (50-100 × 109/L)

Active bleeding with dysfunctional platelets

Front 31

cryoprecipitate, contents and indication?

Back 31

- contains a concentrated source of factor VIII, von Willebrand factor, factor XIII, fibronectin, and fibrinogen

- treatment of choice in bleeding patients with hypofibrinogenemia from liver disease, thrombolytic therapy, or DIC

Front 32

Product of choice to reverse warfarin in life-threatening bleeding?

Back 32

IV vitamin K + Prothrombin Complex Concentrate

Front 33

Transfusion-related Acute Lung Injury, presentation? tx?

Back 33

- hypoxia and dyspnea resembling noncardiac pulmonary edema during or within 6 hours of a transfusion.

- Fever and hypotension, b/l infiltrates with pulmonary edema on CXR.

tx: Intubation may be required, supportive, improve in few days, ban blood donor

Front 34

Febrile Nonhemolytic Transfusion Reaction, management?

Back 34

- stop trf

- rule out AHTR

- resume with close monitoring

- Pretransfusion antipyretics or leukoreduction of cellular blood products may prevent recurrence.

Front 35

Transfusion-associated graft-versus-host disease (T-GVHD)

- who is at risk?

- ppx?

Back 35

- HSCT recipients, recipients of blood transfusion from first-degree relatives, and pt with immunosuppression associated with hematologic malignancies

- ppx with gamma irradiation of blood prdcts

Front 36

severe anaphylactic rxn from RBC trf usually associated with:_____

Management?

Back 36

IgA def pt with anti-IgA Ab

Give washed cells

Front 37

Apheresis

i. What is it?

ii. indications?

iii. monitor for

iv. withhold these before elective procedure:

Back 37

i. separation of whole blood into components, tx and return

ii. sickle cell ACS, malaria, babesia

iii. hypocalcemia

iv. ACE Inhibitor

Front 38

===== BLEEDING DISORDERS ====

Back 38

===== BLEEDING DISORDERS ====

Front 39

Clotting Cascade

Back 39

Front 40

primary hemostasis?

Back 40

injured blood vessel constricts and platelets adhere to TF–bearing cells through interactions between glycoprotein Ib/IX/V and (vWF).

- platelets secrete their granular contents

- Glycoprotein IIb/IIIa undergoes a conformational change, allowing it to bind fibrinogen, which then cross-links platelets

Front 41

secondary hemostasis?

Back 41

- phospholipid scaffold is formed in primary hemo

- prothrombin -> thrombin

- fibrinogen -> fibrin

- 13a X-links fibrin to meshwork

Front 42

A history of bleeding into muscles and joints is characteristic of ?

Back 42

disorders of humoral clotting factors

Front 43

mucosal bleeding occurs more commonly in ?

Back 43

disorders of primary hemostasis

thrombocytopenia

qualitative platelet defects

Front 44

Bleeding associations:

i) enlarged tongue, carpal tunnel syndrome, and periorbital purpura

ii) A harsh systolic murmur

iii) Splenomegaly

Back 44

i) may indicate amyloidosis, which is associated with an acquired deficiency of clotting proteins, especially factor X.

ii) may indicate severe AS, which can cause an acquired type 2 vWD.

iii) can be associated with thrombocytopenia and may indicate underlying cirrhosis.

Front 45

In general, (i) a prolonged PT is most commonly due to ____

(ii) An isolated prolonged aPTT is most commonly due to __

Back 45

i) an acquired deficiency of factor VII, from vitamin K deficiency, liver disease, DIC, or warfarin use

ii) a lupus inhibitor, but hemophilia is also a concern

Front 46

hemophilia A/B are from? tx?

Back 46

Factor 8 and 9 deficiency respectively

tx with concentrates, desmopression for mild Hemo A. No ASA or NSAID

Front 47

normal PTT with

i. giant platelets

ii. abnormal platelet aggregation

Back 47

i. Bernard-Soulier dz

ii. Glanzmann's dz

Front 48

vWD dx, tx?

Back 48

dx: decreased Ristocetin cofactor assay

tx:

mild type 1: desmopressin

severe type 1 or bleeding type 2/3 - vWF-containing factor 8 concentrate

Front 49

acquired hemophilia: lab findings? Assoc with?

Back 49

i) bleeding with an isolated aPTT prolongation, factor 8 low, mixing study won't correct

ii) postpartum state, malignancy, or autoimmune conditions, but 50% of cases are idiopathic

Front 50

acquired hemophilia, tx?

Back 50

if factor 8 inhibitor

titre low: factor 8 concentrate

titre high: factor 7a, PTC to activate factor X, immunosuppresion

Front 51

coagulopathy of liver dz, tx?

Back 51

FFP + vitamin K

Front 52

DIC, lab findings, tx?

Back 52

Low platelet levels, a prolonged PT, a low or decreasing fibrinogen level, and an elevated D-dimer level are diagnostic

- tx: underlying cause + supportive plasma, cryoprecip, plt transfusions

Front 53

Hemophilia A, mild, scheduled for dental extraction, wtd

Back 53

Desmopression spray +/- aminocaproid acid / tranexamic acid

Front 54

Hemophilia A + trauma to head, wtd

Back 54

Give factor 8 concentrate, keep level > 50%