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Project - USMLE Step 3 > Step Up - Hematologic Diseases and Neoplasms > Flashcards

Step Up - Hematologic Diseases and Neoplasms Flashcards

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USMLE® Step 1 flashcards
1
Q

Anemia - Definition:

A

A reduction in Hct or Hb concentration.

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2
Q

When red cell mass decreases, several compensatory mechanisms maintain O2 delivery to the tissues. These include:

A
  1. UP CO.
  2. UP extraction ratio.
  3. Rightward shift of the oxyhemoglobin curve (Incr. 2,3-DPG).
  4. Expansion of plasma curve.
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3
Q

As a general rule, blood transfusion is NOT recommended UNLESS either of the following is true:

A
  1. Hb <7 OR

2. Patient requires incr. O2-carrying capacity - patients with CAD or some other cardiopulmonary disease.

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4
Q

Symptoms of anemia are highly variable, depending on:

A
  1. How rapidly the Ht drops.
  2. Underlying condition of the patient.
  3. Ht level the patient typically lives on.
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5
Q

Pseudoanemia:

A

Refers to a decrease in Hb and Ht 2o to dilution (ie 2o to acute volume infusion or overload).

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6
Q

If Ht and Hb reveals anemia, next tests to obtain to determine the cause of anemia are:

A
  1. Reticulocyte count

2. MCV

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7
Q

All anemias are initially?

A

Normocytic - it takes some time for the abnormal-sized RBCs to outnumber the normal-sized ones.

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8
Q

Anemia of liver disease:

A

MCV up to 115 - due to altered metabolism of plasma lipoproteins into their membranes, altering RBC shape (and incr. volume).

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9
Q

Normocytic anemia - DDX:

A
  1. Aplastic anemia
  2. Bone marrow fibrosis
  3. Tumor
  4. Anemia of chronic disease
  5. Renal failure
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10
Q

If the reticulocyte index is >2, do the following:

A
  1. Suspect blood loss –> Look for the source of bleeding.

2. Suspect hemolysis.

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11
Q

In an anemic patient, 1st step is:

A

To assess the volume status + hemodynamic stability.

If unstable, transfuse PRBCs BEFORE attempting to find the cause of anemia.

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12
Q

Dietary deficiency/incr. iron requirements - primarily seen in:

A
  1. Infants and toddlers.
  2. Adolescents.
  3. Pregnant women.
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13
Q

Microcytic anemias - Serum ferritin:

A

Fe def. –> Low.
Beta thal –> Normal/high.
ACD –> Normal/high.

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14
Q

Microcytic anemias - Serum Iron:

A

Fe def –> Low.
Beta thal –> Normal/high.
ACD –> Low.

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15
Q

Microcytic anemias - TIBC:

A

Fe def. –> High.
Beta thal –> Normal.
ACD –> Normal/low.

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16
Q

Microcytic anemias - RDW:

A

Fe def. –> High.
Beta thal –> Normal/high.
ACD –> Normal.

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17
Q

Treatment of iron def. anemia:

A
  1. Oral iron replacement (ferrous sulfate).
  2. Parenteral iron replacement (iron dextran IV, IM).
  3. Blood transfusion is NOT recommended unless anemia is severe or the patient has cardiopulmonary disease.
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18
Q

What is the main pathogenetic mechanism in beta thal?

A

Excess ALPHA-chains bind to and damage the RBC membrane.

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19
Q

Cooley’s anemia - another name?

A

Beta thal major.

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20
Q

Diagnosis of beta thal major:

A
  1. Hb electrophoresis –> HbF + HbA2 UP.

2. Peripheral smear –> Microcytic/hypochromic, TARGET cells may be seen.

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21
Q

Sideroblastic anemia - Clinical findings:

A
  1. Incr. serum iron + ferritin.
  2. Normal TIBC.
  3. TIBC sat is normal/elevated –> Distinguishes it from Fe def.
  4. Ringed sideroblasts in bone marrow.
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22
Q

Sideroblastic anemia - Treatment:

A

Remove offending agent - Consider pyridoxine.

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23
Q

Aplastic anemia - Can transform into a leukemia?

A

Yes - Into acute leukemia.

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24
Q

Aplastic anemia - Diagnosis:

A
  1. Normocytic/chromic anemia.
  2. Perform a bone marrow biopsy for definitive diagnosis - This reveals HYPOCELLULAR marrow and the absence of progenitors of all 3 hematopoietic cell lines.
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25
Q

Aplastic anemia - Treatment:

A
  1. Bone marrow transplantation.
  2. Transfusion of PRBCs + platelets, if necessary.
  3. Treat any known underlying cause.
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26
Q

VitB12 is involved in 2 important reactions:

A
  1. As a co-factor in conversion of homocysteine to methionine.
  2. As a co-factor in conversion of methylmalonyl CoA to succinyl CoA.
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27
Q

B12 def. due to how much ileac resection?

A

Approx. the last 100cm.

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28
Q

Other organisms competing for B12:

A
  1. Diphyllobothrium latum infestation - Fish tapeworm.

2. Blind-loop syndrome - Bacterial overgrowth.

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29
Q

B12 neuropathy:

A
  1. Demyelination in POSTERIOR columns + Lateral corticospinal + Spinocerebellar tracts –> Loss of position/vibratory sensation in lower extremities + Ataxia and UMN signs.
  2. Can lead to urinary/fecal incontinence, impotence.
  3. Dementia - Investigate in the workup for dementia.
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30
Q

Test that provides info regarding the cause of vitB12 def:

A

Schilling test.

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31
Q

B12 def - Treatment:

A

B12 IM once per MONTH.

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32
Q

What is elevated ONLY in B12 but NOT IN FOLATE def.?

A

Serum MMA!!!

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33
Q

Treatment of folate def. :

A

DAILY oral folate replacement.

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34
Q

The following are relevant in the history of a patient with hemolytic anemia:

A
  1. Ethnic background.
  2. Family history of jaundice/anemia.
  3. Medications.
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35
Q

Lab tests in hemolytic anemia:

A
  1. Elevated reticulocyte count.
  2. Elevated LDH.
  3. Decr. haptoglobin and Hb/Ht..
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36
Q

Dark urine color in intravascular hemolytic anemia:

A

Due to Hburia, NOT bilirubin.

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37
Q

Spherocytes or helmet cells suggest intra- or extra-vascular hemolysis?

A

Extra

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38
Q

Heinz bodies are seen in?

A

G6PD def.

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39
Q

Haptoglobin binds to?

A

Hb

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40
Q

LDH is elevated in hemolytic anemias because?

A

LDH is released when RBCs are destroyed.

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41
Q

Hemolytic anemias - General Treatment:

A
  1. Treat the underlying cause.
  2. Transfusion of PRBCs if severe anemia is present or patient is hemodynamically compromised.
  3. Folate supplements - folate is depleted in hemolysis.
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42
Q

Almost every organ can be affected in SCA:

A
  1. Blood –> Chronic hemolytic anemia, aplastic crises.
  2. Heart –> High-output HF due to anemia.
  3. CNS –> Stroke.
  4. GI tract –> Gallstones, splenic infartions, abdominal crises.
  5. Bones –> Painful crises, osteomyelitis, avascular necrosis.
  6. Lungs –> Infections, acute chest syndrome.
  7. Kidneys –> Hematuria, papillary necrosis, renal failure.
  8. Eyes –> Proliferative retinopathy, retinal infarcts.
  9. Genitalia –> Priapism.
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43
Q

SCA - Prognosis:

A
  1. Survival correlates with the frequency of vaso-occlusive crises - more frequent crises are associated with a shorter lifespan.
  2. If >3 crises per year –> Median age of death is 35.
  3. In general –> Life expectancy is reduced by 25-30yrs.
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44
Q

SCA - Does it require transfusions?

A

The anemia is well compensated and is RARELY transfusion dependent.

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45
Q

SCA - Aplastic crisis - Cause:

A

Usually provoked by a viral infection such as B19 –> Reduces the ability of bone marrow to compensate.

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46
Q

SCA - Aplastic crisis - Treatment:

A

Blood transfusion –> Recovers in 7-10d.

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47
Q

SCA - Painful crises involving bone - How long do they last?

A

2-7days.

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48
Q

SCA - Hand-foot syndrome (dactylitis):

A

Painful swelling of dorsa of hands and feet seen in infancy and early childhood - usually 4-6months.

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49
Q

SCA - What is often the 1st manifestation of SCA?

A

Hand-foot syndrome (dactylitis).

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50
Q

SCA - Hand-foot syndrome - Cause:

A

By avascular necrosis of the metacarpal and metatarsal bones.

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51
Q

SCA - Acute chest syndrome:

A
  1. Repeated pulmonary infarctions.
  2. Clinical presentation is similar to pneumonia.
  3. Associated with chest pain, respiratory distress, pulm. infiltrates, and hypoxia.
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52
Q

SCA - Priapism - How long does it last?

A

Usually 30min to 3hrs.

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53
Q

SCA - Priapism - How does it pass?

A

Usually subsides spontaneously, after urine is passed, after light exercise, or after a cold shower.

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54
Q

Sustained priapism is a?

A

Medical EMERGENCY - Rarely, priapism lasts >3hr.

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55
Q

SCA - Why predisposition for salmonella osteomyelitis?

A

Also due to splenic malfunction.

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56
Q

SCA - Diagnosis:

A
  1. Anemia is the MC finding.
  2. Peripheral smear - sickle-shaped RBCs.
  3. Hb electrophoresis is REQUIRED for diagnosis. In most cases, diagnosis is made from newborn screening tests.
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57
Q

SCA - Advise the patient as follows:

A
  1. Avoid high altitudes (also avoid airplanes).
  2. Maintain fluid intake.
  3. Treat infections properly.
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58
Q

SCA - Management of painful crises:

A
  1. Hydration
  2. Morphine
  3. Keep the patient warm
  4. Supplemental O2 if hypoxia is present
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59
Q

SCA - Treatment - Role of hydroxyurea:

A
  1. Enhances HbF levels - which interferes with the sickling process.
  2. Results in reduced incidence of painful crises.
  3. Accelerates healing of leg ulcers and may reduce recurrence.
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60
Q

SCA - Blood transfusion?

A
  1. Not used unless absolutely necessary.
  2. Not based on Hb levels.
  3. Should be considered in acute chest syndrome, stroke, priapism that does not respond to fluids/analgesia, and cardiac decompensation.
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61
Q

Causes of spherocytosis:

A
  1. Hereditary spherocytosis
  2. G6PD def.
  3. ABO incompatibility (but not Rh incompatibility).
  4. Hyperthermia.
  5. Autoimmune hemolytic anemia.
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62
Q

Hereditary spherocytosis - Diagnosis:

A

RBC osmotic fragility to hypotonic saline.

Also, COOMBS (-) –> Helps to distinguish from AHA, in which spherocytes are also seen!

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63
Q

Mechanism of Heinz bodies formation in G6PD def.?

A

Def. of G6PD –> Accumulation of unneutralized H2O2 –> Denatures Hb –> Precipitates Heinz body formation within RBCs.

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64
Q

Problem with the Heinz bodies?

A

They attach to RBC membranes, reducing their flexibility and making them prone to sequestration by the spleen.

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65
Q

G6PD def. Diagnosis:

A
  1. Peripheral blood smear –> Bite cells, Heinz bodies.
  2. Deficient NADPH formation on G6PD assay.
  3. Measurement of G6PD levels is diagnostic –> HOWEVER, G6PD levels may be NORMAL during the hemolytic episode because the RBCs that are most deficient in G6PD have already been destroyed.
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66
Q

G6PD def - Treatment:

A
  1. Avoid drugs that precipitate hemolysis.
  2. Maintain hydration.
  3. Perform RBC transfusion when necessary.
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67
Q

AHA - Main mechanism:

A

Production of autoantibodies toward RBC membrane antigen(s) which leads to destruction of these RBCs.

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68
Q

AHA - What determines the prognosis, site of RBC destruction, and response to treatment?

A

The type of antibody produced (IgG or IgM).

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69
Q

AHA - Course:

A

Variable - More fulminant in children than in adults.

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70
Q

AHA - Diagnosis - Warm or cold?

A

Direct Coombs test:
If RBCs are coated with IgG –> WAHA.
If RBCs are coated with COMPLEMENT alone –> CAHA.

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71
Q

AHA - Treatment:

A

Often NO TREATMENT is necessary in either type of AHA, because the hemolysis is mild. If it is more severe, the therapeutic approach depends on the type of autoantibody causing the hemolysis.

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72
Q

Warm AHA - Treatment:

A
  1. Mainstay of therapy –> Glucocorticoids.
  2. Splenectomy.
  3. Immunosuppression –> Azathioprine or cyclophosphamide.
  4. RBC transfusions - If absolutely necessary.
  5. Folate supplements.
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73
Q

CAHA - Treatment:

A
  1. Avoid exposure to cold.
  2. RBC transfusion - if absolutely necessary.
  3. Various chemo agents.
  4. Steroids ARE NOT beneficial.
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74
Q

Paroxysmal nocturnal hemoglobinuria (PNH) - What is it?

A

Acquired disorder that affects hematopoietic stem cells and cells of ALL blood lineages.

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75
Q

PNH - Mechanism:

A

Caused by a deficiency of anchor proteins that link complement-inactivating proteins to blood cell membranes.
–> Unusual susceptibility to complement-mediated lysis of RBCs, WBCs, and platelets.

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76
Q

PNH - Thrombosis?

A

YES - of venous systems –> Budd-Chiari syndrome.

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77
Q

PNH - May evolve into?

A
  1. Aplastic anemia.
  2. Myelodysplasia
  3. Myelofibrosis.
  4. Acute leukemia.
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78
Q

PNH - Diagnosis:

A
  1. Ham’s test.
  2. Sugar water test.
  3. Flow cytometry (CD55, CD59).
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79
Q

PNH - Treatment:

A
  1. Glucocorticoids (prednisone) are the usual initial therapy - Many patients do NOT respond.
  2. Bone marrow transplantation.
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80
Q

Heparin-induced thrombocytopenia (HIT) - How many types?

A

2

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81
Q

HIT type 1:

A

Heparin directly causes platelet aggregation - Seen in <48hrs, after initiating heparin - No treatment is needed.

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82
Q

HIT type 2:

A

Heparin induces antibody-mediated injury to platelet - Seen in 3-12days.
Heparin should be discontinued immediately.

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83
Q

Severity of thrombocytopenia and associated risk - >100.000:

A

Abnormal bleeding (even after trauma or surgery) is unusual.

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84
Q

Severity of thrombocytopenia and associated risk - 20.000-70.000:

A

Incr. bleeding hemorrhage during surgery or trauma.

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85
Q

Severity of thrombocytopenia and associated risk - <20.000:

A

Minor spontaneous bleeding, easy bruising, petechiae, epistaxis, menorrhagia, bleeding gums.

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86
Q

Severity of thrombocytopenia and associated risk - <5.000:

A

Major spontaneous bleeding, intracranial bleeding, heavy GI bleeding.

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87
Q

Immune (idiopathic) thrombocytopenic purpura - What happens?

A

Results from autoimmune antibody formation against host platelets. These antiplatelet antibodies (IgG) coat and damage platelets, which are then removed by splenic macrophages.

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88
Q

Immune (idiopathic) thrombocytopenic purpura - 2 forms:

A

Acute and chronic.

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89
Q

Immune (idiopathic) thrombocytopenic purpura - Acute form:

A
  1. Seen in children.
  2. Preceded by viral infection (in most cases).
  3. Usually self-limited - 80% resolve spontaneously within 6 months.
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90
Q

Immune (idiopathic) thrombocytopenic purpura - Chronic form:

A
  1. Usually seen in adults, most commonly in women between 20-40yrs of age.
  2. Spontaneous remissions are rare.
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91
Q

ITP - Clinical features:

A
  1. Petechiae and ecchymoses on the skin - many patients will have only very minimal bleeding symptoms despite extremely low platelet counts (<5.000).
  2. Bleeding of the mucous membranes.
  3. No splenomegaly.
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92
Q

ITP - Diagnosis:

A
  1. Platelet count is frequently <20.000.
  2. Peripheral smear shows decreased platelets.
  3. Bone marrow aspiration shows incr. megakaryocytes.
  4. There is an incr. amount of platelet-associated IgG.
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93
Q

ITP - Treatment:

A
  1. Corticosteroids.
  2. IVIG –> Saturates reticuloendothelial system binding sites.
  3. Splenectomy –> Remission in 70-80% of cases.
  4. 2 new drugs –> Romiplastim, eltrombopag for splenectomy-resistant patients.
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94
Q

Thrombotic thrombocytopenic purpura (TTP) - Mechanism:

A

Rare disorder of platelet consumption. Cause is unknown.

  • -> Hyaline microthrombi (mostly platelet thrombi) occlude small vessels - any organ may be involved.
  • -> Mechanical damage to RBCs –> Schistocytes on peripheral smear.
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95
Q

TTP is a?

A

Life-threatening EMERGENCY that is responsive to therapy. If untreated, death occurs within a few months.

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96
Q

TTP - PT and PTT?

A

There is no consumption of clotting factors, so PT/PTT are normal.

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97
Q

TTP and HUS?

A

TTP = HUS + fever + altered mental status.

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98
Q

HUS is?

A

HUS = Microangiopathic hemolytic anemia + Thrombocytopenia + Renal failure.

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99
Q

TTP - Treatment:

A
  1. Plasmapheresis (large volume) –> ASAP!!!
  2. Corticosteroids and splenectomy - may be of benefit in some cases.
  3. Platelet transfusions are CONTRAindicated.
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100
Q

HIT - Main complications:

A
  1. Pulm. embolism

2. DVT.

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101
Q

What suggests HIT?

A

Decr. in platelet count by 50%.

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102
Q

HIT - Diagnostic tests:

A

Antiplatelet factor IV antibody or serotonin release assay.

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103
Q

HIT treatment:

A
  1. Stop heparin.

2. If anticoagulation is indicated (venous thrombosis), give a thrombin inhibitor such as lepirudin.

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104
Q

Bernard-Soulier syndrome - Inheritance pattern:

A

AR

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105
Q

B-S syndrome - Problem:

A

Disorder of platelet adhesion (to subendothelium) due to deficiency of platelet glycoprotein (GPIb-IX).

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106
Q

B-S syndrome - Peripheral smear:

A

Platelets are abnormally large.

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107
Q

B-S syndrome - Platelet count:

A

Mildly low.

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108
Q

Glanzmann’s thrombasthenia - Inheritance pattern:

A

AR

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109
Q

Glanzmann thrombasthenia - Problem:

A

Disorder of platelet aggregation –> Due to deficiency in platelet glycoprotein GPIIb-IIIa.
Bleeding time is prolonged.
Platelet count is normal.

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110
Q

The ristocetin assay can be used to test for?

A

vWD.

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111
Q

Factor VIII has 2 portions:

A
  1. The coagulant portion - Factor VIII coagulant protein.

2. The antigenic portion - Factor VIII antigenic protein –> Synonymous with vWF.

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112
Q

vWD - What happens?

A

AD disorder characterized by DEFICIENCY or DEFECT of factor VIII-related antigen (vWF).

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113
Q

Role of vWF:

A
  1. Enhances platelet aggregation + adhesion - First steps in clot formation.
  2. It also acts as a carrier of factor VIII in blood.
114
Q

Types of vWD:

A 
Type 1 (MC form) - decr. levels of vWF.
Type 2 (less common) - exhibits qualitative abnormalities of vWF.
Type 3 (least common) - Absent vWF (very severe disease).
115
Q

Mention 3 other causes of impaired platelet function.

A
  1. Uremia
  2. NSAIDs
  3. Aspirin
116
Q

Site of synthesis of vWF:

A
  1. Endothelial cells

2. Megakaryocytes (!)

117
Q

vWF functions:

A
  1. Platelet adhesion - mediates adhesion of platelets to the injured vessel walls.
  2. Binds the factor VIII coagulant protein and protects it from degradation.
118
Q

vWD - Diagnosis:

A
  1. Clinical findings and lab info, which can be variable.
  2. Prolonged bleeding time - Normal platelet count.
  3. Decr. plasma vWF, decr. factor VIII activity.
  4. Reduced ristocetin-induced platelet aggregation (!!).
119
Q

vWD Treatment:

A
  1. DDVAP (desmopressin) - induces endothelial cells to secrete vWF.
  2. Factor VIII concentrates (containing high-molecular-weight vWF).
  3. Cryoprecipitate is NOT recommended as treatment for vWD because it carries the risk of viral transmission.
  4. Avoid aspirin/NSAIDs as well as intramuscular injections - Exacerbate bleeding tendency.
120
Q

First line treatment for vWD is?

A

Desmopressin. If no response, give VIII concentrate.

121
Q

Hemophilia A - Prevalence:

A

1/10.000 male patients.

122
Q

MCC of death in hemophilia A:

A

AIDS due to past history of transfusion, before screening was initiated.
2nd is intracranial bleeding.
–> More than 75% of Hemophilia A patients are HIV(+).

123
Q

Hemophilia A - Diagnosis:

A
  1. Prolonged PTT.

2. Low factor VIII coagulant level + normal levels of vWF.

124
Q

Detection of factor VIII inhibitor:

A
  1. If normal plasma is mixed with plasma from a hemophiliac patient, PTT becomes normal.
  2. If PTT fails to normalize, this is diagnostic of the presence of a factor VIII inhibitor.
125
Q

Thrombin time:

A

Measure of fibrinogen concentration.

126
Q

Bleeding time:

A

Reflects platelet function.

127
Q

Normal PT:

A

11-15sec.

128
Q

Normal PTT:

A

25-40sec.

129
Q

Normal bleeding time:

A

2-7min.

130
Q

DIC etiology:

A
  1. Infection - MCC, especially gram(-) sepsis.
  2. Obstetric complications.
  3. Major tissue injury - Trauma, major surgery, burns, fractures.
  4. Malignancy.
  5. Shock, circulatory collapse.
  6. Snake venom (rattlesnakes).
131
Q

DIC - Clinical features:

A
  1. Bleeding - more common in acute phases.

2. Thrombosis - more common in chronic phases.

132
Q

Whenever a coagulopathy is present, consider vitK def. and liver disease in the DDx (in addition to DIC):

A

Liver disease –> PT and PTT are elevated, but TT, fibrinogen, and platelets are usually normal.
VitK –> PT is prolonged, but PTT, TT, platelet count, fibrinogen level S are normal.

133
Q

Complications of DIC:

A
  1. Hemorrhage - Intracranial bleeding is a common cause of death.
  2. Thromboembolism - Stroke, pulmonary embolism, bowel infarction, ARF, arterial occlusion.
134
Q

DIC - Diagnosis - What does increase?

A

The following are ALL increased:

  1. PT, PTT, bleeding time, TT.
  2. Fibrin split porducts (due to activation of fibrinolytic system).
  3. D-dimer.
135
Q

DIC - Diagnosis - What does decrease?

A
  1. Fibrinogen level (if normal or elevated essentially rules out diagnosis).
  2. Platelet count - thrombocytopenia.
136
Q

DIC - Peripheral smear:

A

Schistocytes from damage of RBCs as they go through the microcirculation (with microthrombi).

137
Q

Platelet count is normal in which bleeding disorders?

A
  1. Hemophilia
  2. vWD
  3. Heparin (normal or decr.)
  4. Warfarin
  5. Liver disease
138
Q

Platelet count is decreased in which bleeding disorders?

A
  1. ITP
  2. TTP
  3. DIC
139
Q

Bleeding time is normal in which bleeding disorders?

A
  1. Hemophilia
  2. Heparin
  3. Warfarin
  4. Liver disease
140
Q

Bleeding time is increased in which bleeding disorders?

A
  1. vWD
  2. ITP
  3. TTP
  4. DIC
141
Q

PT is normal in which bleeding disorders?

A
  1. Hemophilia
  2. vWD
  3. ITP
  4. TTP
  5. Heparin
142
Q

PT is increased in which bleeding disorders?

A
  1. DIC
  2. Warfarin
  3. Liver disease
143
Q

PTT is increased in which bleeding disorders?

A
  1. Hemophilia
  2. vWD
  3. DIC
  4. Heparin
  5. Liver disease
144
Q

PTT is normal in which bleeding disorders?

A
  1. ITP
  2. TTP
  3. Warfarin
145
Q

VitK def. - Factors affected?

A

II, VII, IX, X, C, S.

146
Q

VitK def. etiology:

A
  1. Broad-spectrum antibiotics (suppression of gut flora) in patients who are NPO (inadequate dietary intake).
  2. Patients on TPN (unless vitK is added).
  3. Malabsorption of fat-soluble vitamins.
  4. Warfarin.
147
Q

VitK is most commonly seen in?

A

Critically ill patients - Who are NPO and on antibiotics.

148
Q

VitK def. - Clinical features:

A
  1. Hemorrhage - Serious bleeding can develop.
  2. PT is initially prolonged (factor VII has the shortest half-life).
  3. PTT prolongation follows (as other factors diminish).
149
Q

VitK def. - Treatment:

A
  1. VitK replacement (oral or SC) - May take a few days for PT to return to normal.
  2. If bleeding is severe and emergency treatment is necessary, FFP should be transfused.
150
Q

All clotting factors are produced by the liver EXCEPT?

A

vWF

151
Q

The following are the reasons coagulopathy develops in liver failure:

A
  1. Decr. synthesis of clotting factors.
  2. Cholestasis leads to decr. vitK absorption.
  3. Hypersplenism –> Thrombocytopenia.
152
Q

ATIII def. - Inheritance pattern:

A

AD

153
Q

Patients with ATIII def. do NOT respond to?

A

HEPARIN

154
Q

Antiphospholipid antibody syndrome - Antibodies:

A

Antibodies against:

  1. Lupus anticoagulant.
  2. Anticardiolipin.
  3. β2-microglobulin.
155
Q

Protein C deficiency - Inheritance pattern:

A

AD

156
Q

Protein C def - What happens?

A

It is an inhibitor of factors V and VIII –> Unregulated fibrin synthesis.

157
Q

Protein S def?

A

Protein S is a cofactor of protein C, so a deficiency leads to decr. protein C activity.

158
Q

Factor V leiden - What happens?

A

Activated protein C resistance - A mutation in factor V gene –> Protein C can no longer inactivate factor V –> Unregulated prothrombin activation, and thus an increase in thrombotic events.

159
Q

Secondary hypercoagulable states or risk factors:

A
  1. Malignancy (esp. pancreas, GI, lung, ovaries).
  2. Antiphospholipid antibody syndrome.
  3. Pregnancy - up to 2 months post partum.
  4. Immobilization, causing stasis of blood.
  5. Myeloproliferative disorders.
  6. OCPs
  7. Post-op states (esp. after orthopedic procedures).
  8. Trauma
  9. Nephrotic syndrome.
  10. HIT or DIC.
  11. PNH.
  12. HF - Stasis of blood.
160
Q

Suspect an inherited hypercoagulable state if one or more of the following are present:

A
  1. Patient has a history of DVT, PE, or thrombotic events.
  2. Patient has recurrent episodes of DVT, PE, or thrombotic events.
  3. Patient’s first thrombotic event was before age 40.
  4. Patient experiences thrombosis in unusual sites (eg in mesenteric veins, IVC, renal veins, or cerebral veins).
161
Q

Inherited hypercoagulable states - Diagnosis:

A

Functional assays are available for:

  1. Antithrombin
  2. Antiphospholipid antibodies
  3. Protein C
  4. Protein S
  5. Factor V leiden
  6. Prothrombin gene mutation
  7. Hyperhomocysteinemia
162
Q

Heparin - Mechanism of action:

A
  1. Potentiates the action of antithrombin to primarily inhibit clotting factors IIa and Xa.
  2. Prolongs PTT.
  3. Half-life of standard heparin is 1h. Longer for LMWHs.
163
Q

Option for DVT prophylaxis:

A
  1. LMWH.
  2. Low-Dose unfractionated heparin.
  3. Pneumatic compression boots.
164
Q

Heparin - Indications for use:

A
  1. Venous thromboembolism: DVT, PE.
  2. Acute coronary syndrome: unstable angina, MI.
  3. DVT (Low dose, or LMWH).
  4. A-fib in acute setting.
  5. After vascular bypass grafting.
165
Q

How is therapeutic heparin now often monitored?

A

Using antifactor Xa levels.

166
Q

Heparin - Side effects:

A
  1. Bleeding
  2. HIT - lower incidence with LMWHs.
  3. Possible osteoporosis - Lower incidence with LMWHs.
  4. Transient alopecia.
  5. Rebound hypercoagulability after removal due to depression of ATIII.
167
Q

Contraindications to heparin:

A
  1. Previous HIT
  2. Active bleeding
  3. GI bleeding
  4. Intracranial bleeding
  5. Hemophilia, thrombocytopenia
  6. Severe HTN (!).
  7. Recent surgery on eyes, spine, brain.
168
Q

LMWHs - Mechanism of action:

A

Mostly inhibit factor Xa, but LESS inhibition of factor IIa (thrombin) and platelet aggregation.

169
Q

LMWHs - Indications for use:

A
  1. Only SC, not IV administration.
  2. PTT monitoring is NOT necessary.
  3. Excreted via the kidneys - Use cautiously in patients with renal dysfunction.
  4. Much more expensive than standard heparin.
170
Q

Warfarin - Side effects:

A
  1. Hemorrhage
  2. Skin necrosis is a rare but serious complication. It is caused by rapid decrease in protein C (a vitK-dependent inhibitor of factors Va and VIIIa.
  3. Teratogenic - avoid during pregnancy.
  4. Should NOT be given to alcoholics or to any patient who is prone to frequent falls because an intracranial bleed in a patient on warfarin can be catastrophic.
171
Q

Reversing the effects of warfarin:

A
  1. Discontinue warfarin and administer vitK.
  2. Half-life of warfarin is much longer than that of heparin - VitK infusion corrects an abnormal PT within 4 to 10hrs if the patient has a normal liver function.
  3. Giving vitK makes it difficult to return the patient to therapeuticINR levels if anticoagulation is to be continued.
172
Q

If rapid reversal of acute bleeding from warfarin is indicated, give?

A

FFP.

173
Q

Clopidogrel - Mechanism of action:

A
  1. Blocks binding of ADP to a specific ADP receptor P2Y12, which reduces platelet activation and aggregation.
  2. Incr. bleeding time.
174
Q

Which drugs should not be given with clopidogrel?

A

PPIs.

175
Q

Oncologic emergencies:

A
  1. Hypercalcemia - IV fluids, diuretics, bisphosphonates.
  2. Spinal cord compression - steroids, get an MRI.
  3. Pericardial tamponade - pericardiocentesis.
  4. Tumor lysis syndrome (IV fluids, treat individual electrolyte abnormalities).
176
Q

MGUS - Prevalence?

A

Common in elderly - Up to 10% in patients >75yrs of age.

177
Q

MGUS - Diagnosis:

A
  1. IgG spike <1g/24h.

4. There should also be NO end-organ damage (lytic bone lesions, anemia, hypercalcemia).

178
Q

MGUS - Course:

A
  1. Fewer than 20% develop MM in 10-15yrs. Several studies have shown that almost ALL PATIENTS with MM had a preceding MGUS.
  2. No specific treatment is necessary.
179
Q

Signs of MM - Mnemonic CRAB:

A
  1. Calcium (hypercalcemia).
  2. Renal failure
  3. Anemia
  4. Bone lesions
180
Q

4 features that suggest MM:

A
  1. Low Hb.
  2. High Ca.
  3. High serum protein.
  4. Poor renal function.
181
Q

Diagnostic criteria for MM:

A

AT LEAST 10% abnormal plasma cells in bone marrow +

  1. M-protein in the serum.
  2. M-protein in the urine.
  3. Lytic bone lesions - Predominantly found in the skull and axial skeleton.
182
Q

MCC of death in MM:

A

Infections - Up to 70%.

183
Q

MM - Prognosis:

A

5yr survival is 10%.

184
Q

Waldenstrom macroglobulinemia - What happens?

A

Malignant proliferation of plasmacytoid lymphocytes –> Produce IgM paraprotein, which is very large and causes hyperviscosity of the blood.

185
Q

Walderstrom macroglobulinemia - Diagnosis:

A
  1. IgM>5g/dL.
  2. Bence-Jones proteinuria in 10% of cases.
  3. Absence of bone lesions.
186
Q

Waldenstrom macroglobulinemia - Clinical features:

A
  1. Fatigue
  2. Weight loss
  3. Neurologic symptoms
  4. Lymphadenopathy
  5. Splenomegaly
  6. Anemia
  7. Abnormal bleeding
  8. Hyperviscosity due to IgM
187
Q

Waldenstrom macroglobulinemia - Treatment:

A

There is NO DEFINITIVE CURE - Use chemo + plasmapheresis for hyperviscosity syndrome.

188
Q

Hyperviscosity syndrome can lead to ?

A

Retinal vessel dilation with resulting hemorrhage and possible blindness.

189
Q

Ann Harbor staging system for HL:

A

Stage I –> Confined to single lymph node.
Stage II –> Involvement of two or more lymph nodes but confined to same side of diaphragm.
Stage III –> Both sides of diaphragm.
Stage IV –> Dissemination of disease to extralymphatic sites.
A–> No symptoms.
B–> Fever, weight loss, night sweats (presence of these constitutional symptoms worsens the prognosis).

190
Q

Cure in HL:

A

Chemo and radiation therapy in combination achieve cure rates of over 70%.

191
Q

Key epidemiological associations with NHL:

A
  1. Burkitt’s lymphoma in regions of Africa.
  2. Patients with HIV and HIV-associated lymphomas.
  3. Adult T-cell lymphoma in Japan and the Caribbean.
192
Q

HL Diagnosis - What is required?

A

Lymph node biopsy - Presence of RS cells is REQUIRED to make the diagnosis.
+ Presence of inflammatory cells reactive to the RS cells.

193
Q

HL treatment:

A
  1. Stages I, II, and IIIA can be treated with radiotherapy alone. However, some physicians advocate the use of chemo in these patients as well.
  2. Stages IIIB and IV require chemo.
194
Q

Rituximab is?

A

A monoclonal antibody against CD20 antigen.

195
Q

NHL or HL is more common?

A

NHL is TWICE as common as HL.

196
Q

6th MCC of cancer-related death in the US:

A

NHL

197
Q

Risk factors for NHL:

A
  1. HIV/AIDS
  2. Immunosuppression (organ transplant recipients).
  3. History of certain viral infections (EBV, HTLV-1).
  4. History of H.pylori gastritis (maltoma).
  5. Autoimmune - Sjogren, Hashimoto.
198
Q

NHL - Clinical features:

A
  1. Lymphadenopathy.
  2. B symptoms - LESS common than in HL.
  3. HSM, abdominal pain, fullness.
  4. Recurrent infections, symptoms of anemia, or thrombocytopenia due to BM involvement.
  5. Various findings are possible - SVC obstruction, respiratory involvement, bone pain, skin lesions.
199
Q

NHL - Diagnosis - Lymph node biopsy:

A

For DEFINITIVE diagnosis - Any lymph node >1cm present for >4weeks that cannot be attributed to infection should be biopsied.

200
Q

NHL - Diagnosis - Other tests that may help in diagnosis:

A
  1. CXR - Hilar/mediastinal adenopathy.
  2. CT scan - To determine the extent of disease spread and patient’s response to treatment.
  3. Serum LDH and β2-microglobulin –> Indirect indicators of tumor burden.
  4. If ALP is elevated –> Bone/liver involvement is likely.
  5. LFTs.
  6. CBC.
  7. Serum electrolytes, renal function tests.
  8. BM biopsy.
201
Q

Low grade lymphomas - Prognosis:

A
  1. Cure is rare.

2. Median survival is 5-7yrs.

202
Q

Intermediate-grade lymphomas - Prognosis:

A

50% can be cured with aggressive therapy - Median survival is about 2yrs.

203
Q

High-grade lymphomas - Prognosis:

A

Up to 70% can be cured with aggressive therapy - Median survival WITHOUT treatment is a few months.

204
Q

CHOP therapy consists of:

A

Cyclophosphamide
Hydroxydaunomycin (doxorubicin)
Oncovin (vincristine)
Prednisone

205
Q

Percentages for leukemias:

A

Acute leukemias account for 60% of all leukemias.
25% are CLL.
15% are CML.

206
Q

General evaluation in patients with leukemias (acute or chronic):

A

Evaluate patient for:

  1. Evidence of infection.
  2. Evidence of bleeding or easy bruising.
  3. Lymphadenopathy, HSM.
  4. Signs of anemia.
  5. Fatigue, weight loss.
207
Q

Testicular involvement is a feature of which leukemia?

A

ALL

208
Q

Anterior mediastinal mass is a feature of which leukemia?

A

T-ALL

209
Q

Skin nodules are a feature of which leukemia?

A

AML

210
Q

Tumor lysis syndrome:

A
  1. Potential complication of chemo seen in acute leukemia and high-grade NHL.
  2. Rapid cell death with release of intracellular contents causes –> Hyperkalemia, hyperphosphatemia, hyperuricemia.
  3. Treat as medical emergency.
211
Q

Leukemias - Treatment of emergencies:

A
  1. Blood cultures, antibiotics for infections.
  2. Blood transfusion for anemia.
  3. Platelet transfusion for bleeding, if necessary.
212
Q

How is CLL usually discovered?

A

On a routine CBC - Lymphocytosis.

213
Q

AHA can be seen in patients with which type of leukemia?

A

CML

214
Q

Remember that the cells of the myeloid line are:

A
  1. Erythrocytes
  2. Granulocytes
  3. Platelets
215
Q

CLL - WBC:

A

50.000-200.000.

216
Q

Differentiating benign leukemoid reaction from CML leukemoid reaction:

A
  1. Usually no splenomegaly.
  2. Incr. leukocyte ALP.
  3. History of precipitating event.
    CML –> OPPOSITE to the above.
217
Q

CML without the Philadelphia chromosome - Prognosis:

A

WORSE than those with the Phil chromosome.

218
Q

CML - Most patients present:

A

In the chronic phase (85%).

May be ASYMPTOMATIC at the time of diagnosis - disease discovered on routine blood work.

219
Q

CML - Diagnosis - Lab findings:

A
  1. Marked leukocytosis - WBCs from 50.000 to 200.000 with a LEFT shift toward granulocytes.
  2. Small numbers of blasts and promyelocytes.
  3. Eosinophilia.
220
Q

CML - Diagnosis - Leukocyte ALP activity is INCREASED or DECREASED?

A

DECREASED!

221
Q

CML - Diagnosis - Thrombocytosis or thrombocytopenia?

A

Thrombocytosis.

222
Q

Polycythemia vera - Symptoms due to hyperviscosity:

A
  1. Headache
  2. Dizziness
  3. Weakness
  4. PRURITUS
  5. Visual impairment
  6. Dyspnea
223
Q

PV - Thrombotic phenomena:

A
  1. DVT
  2. CVA
  3. MI
  4. Portal vein thrombosis
224
Q

PV - Bleeding:

A
  1. GI or GU bleeding
  2. Ecchymoses
  3. Epistaxis
225
Q

PV - Other features:

A
  1. HSM

2. HTN (!)

226
Q

PV - Diagnosis - 1st step.

A

Rule out causes of 2o polycythemia (hypoxemia, CO exposure).

227
Q

PV - Diagnostic criteria - Major criteria:

A
  1. Elevated RBC mass (men >36L/kg; women>32L/kg).
  2. SaO2>92%.
  3. Splenomegaly.
228
Q

PV - Diagnostic criteria - Minor criteria:

A
  1. Thrombocytosis.
  2. Leukocytosis.
  3. Leukocyte ALP >100 (no fever, no infection).
  4. Serum vitB12 >900.
229
Q

PV - Treatment:

A

Repeated phlebotomy to lower Ht.

230
Q

Myodysplastic syndromes - Definition:

A
  1. A class of acquired clonal blood disorders.
  2. Ineffective hematopoiesis.
  3. Apoptosis of myeloid precursors.
  4. Pancytopenia, despite a normal or hypercellular bone marrow.
231
Q

Myelodysplastic syndromes - Target:

A

More commonly in elderly patients - slightly more common in men.

232
Q

Myelodysplastic syndromes - Etiology:

A
  1. Usually idiopathic.

2. Radiation, immunosuppression, certain toxins.

233
Q

Myelodysplastic syndromes - Prognosis:

A

Although variable, is generally POOR, and the end result is often progression to acute leukemia.

234
Q

Myelodysplastic syndromes - Clinical features:

A
  1. Often ASYMPTOMATIC in the early stages. Pancytopenia may be an incidental finding on a routine blood test.
  2. May present with manifestations of anemia/neutropenia/thrombocytopenia.
    - -> Mimic aplastic anemia
235
Q

MDS - Diagnosis - Biopsy?

A

BM biopsy –> dysplastic cells with blasts or ringed sideroblasts.

236
Q

MDS - Diagnosis - CBC with peripheral smear:

A
  1. Normal/Mildly elevated MCV.
  2. LOW reticulocyte count.
  3. Howell-Jolly bodies, basophilic stippling, nucleated RBCs, hypolobulated neutrophilic nuclei, large, agranular platelets.
237
Q

Howell-Jolly bodies:

A

DNA remnants inside RBCs –> Indicate problem with the spleen.

238
Q

MDS - Treatment:

A
  1. Mainly supportive.
  2. RBC and platelet transfusion are the mainstays of treatment.
  3. EPO may help reduce transfusions.
  4. G-CSF for neutropenic patients.
  5. Vitamin supplementation, particularly with vitamins B6, B12, folate.
239
Q

Essential thrombocythemia - Definition:

A

> 600.000/mm3.

240
Q

Essential thrombocythemia - Diagnosis:

A

Diagnosis of exclusion - Reactive thrombocytosis (due to infection, inflammation, bleeding, and so on) and other myeloproliferative disorders must be excluded.

241
Q

Essential thrombocythemia - Clinical features:

A
  1. Primarily manifested by thrombosis.
  2. High morbidity, but LOW mortality.
  3. Splenomegaly, pseudohyperkalemia, elevated bleeding time.
  4. Erythromelalgia - burning pain and erythema of the extremities due to microvascular occlusions.
242
Q

Essential thrombocythemia - Lab:

A

Peripheral smear –> Hypogranular, abnormally shaped platelets.
Bone marrow biopsy –> Incr. number of megakaryocytes.

243
Q

Essential thrombocythemia - Treatment:

A
  1. Antiplatelet agents such as anagrelide and low-dose aspirin.
  2. Hydroxyurea is sometimes used for severe thrombocytosis.
244
Q

NHL - Clinical features:

A
  1. Lymphadenopathy.
  2. B symptoms - LESS common than in HL.
  3. HSM, abdominal pain, fullness.
  4. Recurrent infections, symptoms of anemia, or thrombocytopenia due to BM involvement.
  5. Various findings are possible - SVC obstruction, respiratory involvement, bone pain, skin lesions.
245
Q

NHL - Diagnosis - Lymph node biopsy:

A

For DEFINITIVE diagnosis - Any lymph node >1cm present for >4weeks that cannot be attributed to infection should be biopsied.

246
Q

NHL - Diagnosis - Other tests that may help in diagnosis:

A
  1. CXR - Hilar/mediastinal adenopathy.
  2. CT scan - To determine the extent of disease spread and patient’s response to treatment.
  3. Serum LDH and β2-microglobulin –> Indirect indicators of tumor burden.
  4. If ALP is elevated –> Bone/liver involvement is likely.
  5. LFTs.
  6. CBC.
  7. Serum electrolytes, renal function tests.
  8. BM biopsy.
247
Q

Low grade lymphomas - Prognosis:

A
  1. Cure is rare.

2. Median survival is 5-7yrs.

248
Q

Intermediate-grade lymphomas - Prognosis:

A

50% can be cured with aggressive therapy - Median survival is about 2yrs.

249
Q

High-grade lymphomas - Prognosis:

A

Up to 70% can be cured with aggressive therapy - Median survival WITHOUT treatment is a few months.

250
Q

CHOP therapy consists of:

A

Cyclophosphamide
Hydroxydaunomycin (doxorubicin)
Oncovin (vincristine)
Prednisone

251
Q

Percentages for leukemias:

A

Acute leukemias account for 60% of all leukemias.
25% are CLL.
15% are CML.

252
Q

General evaluation in patients with leukemias (acute or chronic):

A

Evaluate patient for:

  1. Evidence of infection.
  2. Evidence of bleeding or easy bruising.
  3. Lymphadenopathy, HSM.
  4. Signs of anemia.
  5. Fatigue, weight loss.
253
Q

Testicular involvement is a feature of which leukemia?

A

ALL

254
Q

Anterior mediastinal mass is a feature of which leukemia?

A

T-ALL

255
Q

Skin nodules are a feature of which leukemia?

A

AML

256
Q

Tumor lysis syndrome:

A
  1. Potential complication of chemo seen in acute leukemia and high-grade NHL.
  2. Rapid cell death with release of intracellular contents causes –> Hyperkalemia, hyperphosphatemia, hyperuricemia.
  3. Treat as medical emergency.
257
Q

Leukemias - Treatment of emergencies:

A
  1. Blood cultures, antibiotics for infections.
  2. Blood transfusion for anemia.
  3. Platelet transfusion for bleeding, if necessary.
258
Q

How is CLL usually discovered?

A

On a routine CBC - Lymphocytosis.

259
Q

AHA can be seen in patients with which type of leukemia?

A

CML

260
Q

Remember that the cells of the myeloid line are:

A
  1. Erythrocytes
  2. Granulocytes
  3. Platelets
261
Q

CLL - WBC:

A

50.000-200.000.

262
Q

Differentiating benign leukemoid reaction from CML leukemoid reaction:

A
  1. Usually no splenomegaly.
  2. Incr. leukocyte ALP.
  3. History of precipitating event.
    CML –> OPPOSITE to the above.
263
Q

CML without the Philadelphia chromosome - Prognosis:

A

WORSE than those with the Phil chromosome.

264
Q

CML - Most patients present:

A

In the chronic phase (85%).

May be ASYMPTOMATIC at the time of diagnosis - disease discovered on routine blood work.

265
Q

CML - Diagnosis - Lab findings:

A
  1. Marked leukocytosis - WBCs from 50.000 to 200.000 with a LEFT shift toward granulocytes.
  2. Small numbers of blasts and promyelocytes.
  3. Eosinophilia.
266
Q

CML - Diagnosis - Leukocyte ALP activity is INCREASED or DECREASED?

A

DECREASED!

267
Q

CML - Diagnosis - Thrombocytosis or thrombocytopenia?

A

Thrombocytosis.

268
Q

Polycythemia vera - Symptoms due to hyperviscosity:

A
  1. Headache
  2. Dizziness
  3. Weakness
  4. PRURITUS
  5. Visual impairment
  6. Dyspnea
269
Q

PV - Thrombotic phenomena:

A
  1. DVT
  2. CVA
  3. MI
  4. Portal vein thrombosis
270
Q

PV - Bleeding:

A
  1. GI or GU bleeding
  2. Ecchymoses
  3. Epistaxis
271
Q

PV - Other features:

A
  1. HSM

2. HTN (!)

272
Q

PV - Diagnosis - 1st step.

A

Rule out causes of 2o polycythemia (hypoxemia, CO exposure).

273
Q

PV - Diagnostic criteria - Major criteria:

A
  1. Elevated RBC mass (men >36L/kg; women>32L/kg).
  2. SaO2>92%.
  3. Splenomegaly.
274
Q

PV - Diagnostic criteria - Minor criteria:

A
  1. Thrombocytosis.
  2. Leukocytosis.
  3. Leukocyte ALP >100 (no fever, no infection).
  4. Serum vitB12 >900.
275
Q

PV - Treatment:

A

Repeated phlebotomy to lower Ht.

276
Q

Myodysplastic syndromes - Definition:

A
  1. A class of acquired clonal blood disorders.
  2. Ineffective hematopoiesis.
  3. Apoptosis of myeloid precursors.
  4. Pancytopenia, despite a normal or hypercellular bone marrow.
277
Q

Myelodysplastic syndromes - Target:

A

More commonly in elderly patients - slightly more common in men.

278
Q

Myelodysplastic syndromes - Etiology:

A
  1. Usually idiopathic.

2. Radiation, immunosuppression, certain toxins.

279
Q

Myelodysplastic syndromes - Prognosis:

A

Although variable, is generally POOR, and the end result is often progression to acute leukemia.

280
Q

Historical findings to consider in patients with anemia:

A
  1. FHx –> Hemophilia, G6PD def., Thalassemia.
  2. Bleeding - Melena, recent trauma/surgery, hematemesis.
  3. Chronic illnesses –> Renal failure.
  4. Alcoholism –> Folate/B12/Fe def.

Project - USMLE Step 3 (82 decks)

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