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Internal Medicine > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

Definition of Anemia

A

Reduction in Hct or Hgb

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2
Q

Compensatory Mechanisms to Maintain O2 Delivery to Tissue

A
  1. ) Increased SV or CO/HR
  2. ) Increased Extraction Ratio
  3. ) Rightward shift on oxyhemoglobin curve
  4. ) Expansion of Plasma Volume
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3
Q

When is a transfusion needed?

A
  1. Hgb <7

2. Patient requires increased O2 carrying capacity (CAD or cardiopulmonary disease)

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4
Q

Symptoms of anemia (specific and nonspecific)

A

Nonspecific: fatigue, poor concentration, diarrhea, headache, nausea, vague abdominal discomfort, hypotension, tachycardia, pallor (best if seen in conjunctiva)
Specific: jaundice if hemolytic anemia, blood in stool if GI bleed

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5
Q

1 unit of PRBCs increases Hgb by?

A

1 point of Hgb, 3 points of Hct

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6
Q

If H/H reveals anemia, what is the next best step?

A

Get a retic count and MCV

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7
Q

Effective Erythropoeisis Depends on:

A
  1. ) Adequate raw materials: folate, B12, Iron
  2. ) Absence of intrinsic bone marrow disease
  3. ) Adequate EPO release from kidney
  4. ) Survival of reticulocytes (no destruction prior to leaving the bone marrow).
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8
Q

What does it mean if retic count is >2%

A

excessive RBC production or blood loss. The bone marrow is responding adequately to the cause of anemia.

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9
Q

What does it mean if retic count is <2%

A

Inadequate RBC production by bone marrow

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10
Q

Why do you mix PRBCs with Normal Saline and not Lactated Ringers?

A

Because the calcium in LR causes coagulation within the IV lines.

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11
Q

What should you check after a transfusion of PRBCs is completed?

A

CBC

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12
Q

What does FFP contain?

A

All of the clotting factors and no WBCs/RBCs.

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13
Q

Why do you give FFP?

A

Coagulopathy, high PT/PTT, deficiency of clotting factors, liver failure (since vitamin K won’t work)

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14
Q

What should you check after infusion of FFP?

A

PT/PTT

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15
Q

What does cryoprecipitate contain

A

Factor VIII and Fibrinogen

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16
Q

Why do you give cryoprecipitate?

A

Hemophilia A, von Willebrand Disease, or DIC (due to decreased fibrinogen)

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17
Q

How much will 1 unit of platelets raise the platelet count by?

A

10,000

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18
Q

Is acute hemolytic reaction intravascular or extravascular hemolysis?

A

Intravascular: Caused by ABO mismatched blood transfused into the patient. IgM antibodies attach to the infused RBCs, activate a complement pathway, and produce a massive intravascular hemolysis as C9 punches holes in the RBC membrane.

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19
Q

Symptoms of Acute Hemolytic Reaction

A

N/V, fever, chills, pain in flank/back, chest pain, dyspnea

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20
Q

Complications of Acute Hemolytic Reaction

A

DIC, renal failure w/ hemoglobinuria, hypovolemic shock with hypotension and tachycardia

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21
Q

How do you treat/manage Acute Hemolytic Reaction?

A
  1. Stop the transfusion!!!
  2. Aggressive fluid replacement to prevent renal failure and shock
  3. Epinephrine for anaphylaxis
  4. Dopamine/NE as needed for pressors/to maintain BP
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22
Q

Is delayed hemolytic transfusion reaction intravascular or extravascular?

A

Extravascular.

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23
Q

What causes a delayed hemolytic transfusion reaction

A

Can occur 3-4 days after transfusion. It occurs when one of the minor RBC antigens is found in the transfusion. Reexposure of memory B cells to the antigen on RBCs after prior exposure results in the synthesis of IgG anti-Kell (for ex) antibodies. These antibodies coat the antigen positive RBC donor cells which will be removed extravascularly by macrophages in the liver and bone marrow.

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24
Q

Symptoms of delayed hemolytic transfusion reaction

A

fever, jaundice anemia

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25
Q

How do you treat/manage delayed hemolytic transfusion reaction?

A

Don’t do anything. The prognosis is good and is usually self limited.

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26
Q

Definition of Microcytic Anemia

A

MCV <80

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27
Q

What is in the differential diagnosis of a microcytic anemia?

A
  1. ) Iron deficiency anemia
  2. ) Anemia of Chronic Disease
  3. ) Thalassemias
  4. ) Ring Sideroblastic Anemias (lead poisoning, pyridoxine deficiency, toxic effects of alcohol)
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28
Q

Definition of Macrocytic Anemia

A

MCV >100

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29
Q

What is in the differential diagnosis of macrocytic anemia?

A
  1. ) Nuclear defect: Folate or B12 deficiency
  2. ) Liver disease
  3. ) Stimulated erythropoiesis
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30
Q

How does liver disease lead to a macrocytic anemia?

A

MCV can get up to 115! This is due to altered metabolism of plasma lipoproteins into their membranes altering the shape of RBCs and therefore their volume.

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31
Q

What is in the differential diagnosis of a normocytic anemia?

A
  1. ) Aplastic anemia
  2. ) Bone marrow fibrosis
  3. ) tumor
  4. ) Anemia of Chronic Disease
  5. ) Renal Failure 2/2 to decreased EPO production
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32
Q

If the Retic index is >2% what should you do?

A
  1. ) Consider blood loss and look for the source.

2. ) Consider hemolysis and check: LDH, haptoglobin, bilirubin levels

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33
Q

What are the 2 most common causes of iron deficiency anemia worldwide?

A
  1. ) Chronic blood loss

2. ) Increased iron requirements/dietary deficiency

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34
Q

What are the 2 most common causes of chronic blood loss leading to anemia?

A
  1. ) Menstrual blood loss

2. ) GI bleeding

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35
Q

What populations require increased iron or are at risk of dietary deficiency of iron?

A
  1. ) Infants and toddlers, particularly those who are on human breast milk
  2. ) Teenagers, especially menstruating girls
  3. ) Pregnant women
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36
Q 
Diagnostic values of the following for iron deficiency anemia: 
Ferritin
TIBC/Transferrin levels 
TIBC Saturation 
Serum Iron 
Peripheral blood smear
A 
Ferritin: decreased 
TIBC/Transferrin levels: increased
TIBC Saturation: decreased 
Serum Iron: decreased 
Peripheral blood smear: microcytic hypochromic RBCs
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37
Q

Treatment of Iron Deficiency Anemia:

A
  1. ) In menstruating females try a trial of oral ferrous sulfate.
  2. ) in men and postmenopausal women attempt to determine the source of bleeding
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38
Q

Describe the pathophysiology of beta-thalassemia

A

beta chain production is deficient but alpha chain production is normal. Excess alpha chains bind to and damage the RBC membrane.

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39
Q

Describe the pathophysiology of alpha thalassemia

A

There is a decrease in the alpha chain production. The excess beta chains form tetramers which are abnormal hemoglobins.

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40
Q

What is a complication of the treatment of thalassemia major?

A

Iron overload because these patients are often transfusion dependent

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41
Q

If iron deficiency anemia is suspected but it does not respond to oral iron therapy, what is the next best step?

A

obtain a hemoglobin electrophoresis to rule out alpha or beta thalassemia

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42
Q

What are the clinical features of thalassemia major/Cooley anemia/homozygous beta-chain thalassemia?

A
  • Severe anemia that is microcytic and hypochromic
  • Massive hepatosplenomegaly
  • Expansion of the marrow space which can cause distortion of bones
  • Growth retardation and FTT
  • Progressive CHF if left untreated
  • Crew cut appearance of skull on XR.
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43
Q

What is seen on the hemoglobin electrophoresis of a patient with thalassemia major? Peripheral blood smear?

A
  1. Elevated HbF and HbA2.

2. Hypochromic microcytic cells; target cells

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44
Q

What are the clinical features of thalassemia minor/heterozygous beta-chain thalassemia?

A

these patients are usually asymptomatic and usually require no treatment.

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45
Q

What is the genotype of a silent carrier of alpha thalassemia?

A

These patients have a mutation/deletion of only ONE of the alpha-locus.
They are asymptomatic with a normal H/H and require no treatment.

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46
Q

What is the genotype of alpha-thalassemia trait or alpha thalassemia minor?

A

These patients have a mutation/deletion of TWO of the alpha locus.

They may have a microcytic hypochromic anemia and usually require no treatment. Common in AAs.

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47
Q

What is the genotype of HbH disease?

A

This is hemoglobin Barts disease. These patients have mutation/deletion of THREE of the alpha loci.
Hemoglobin electrophoresis will show HbH. Treatment is transfusion, sometimes splenectomy is helpful.

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48
Q

What is the genotype of alpha thalassemia major?

A

This is a mutation/deletion of all FOUR alpha loci. These patients often have hydrops fetalis and die shortly after birth.

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49
Q

What is the most common type of thalassemia?

A

Thalassemia minor. Beta-thalassemia minor is more common than alpha-thalassemia minor.

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50
Q 
What are the lab results of the following for sideroblastic anemia?
Ferritin 
TIBC/Transferrin
TIBC Saturation
Serum iron
A

Ferritin: increased
TIBC/Transferrin: normal TIBC
TIBC Saturation: normal/elevated
Serum iron: increased

Ringed sideroblasts in bone marrow

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51
Q

What is the treatment for sideroblastic anemia

A
  1. ) Remove the offending agent

2. ) Consider pyridoxine

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52
Q

What are some of the acquired causes of sideroblastic anemia

A
  1. ) Drugs: chloramphenicol, INH, alcohol
  2. ) Lead exposure
  3. ) Collagen vascular disease
  4. ) Neoplastic disease: myelodysplastic syndromes.
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53
Q

What is the pathophysiology of sideroblastic anemia?

A

It is caused by an abnormality of iron metabolism in RBCs.

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54
Q

Why does chronic inflammation lead to anemia?

A

It is thought that inflammatory cytokines have a suppressive effect on erythropoiesis

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55
Q 
What are the lab results of the following for anemia of chronic disease?
Ferritin 
TIBC/Transferrin
TIBC Saturation
Serum iron
A 
Ferritin: increased
TIBC/Transferrin: low
TIBC Saturation: high 
Serum iron: low 
Normocytic and normochromic
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56
Q

What are the causes of aplastic anemia?

A
  1. ) Idiopathic
  2. ) Viral: parvovirus, Hep C, Hep B, EBV, CMV, Herpes zoster varicella, HIV.
  3. ) Malignancy
  4. ) Radiation exposure
  5. ) Medications: chloramphenicol, sulfonamides, gold, carbamazepine
  6. ) Chemicals: benzene, insecticides.
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57
Q

What are the clinical features of aplastic anemia?

A
  1. ) fatigue and dyspnea due to anemia
  2. ) Increased risk of infection due to neutropenia
  3. ) Petechiae, bruising due to thrombocytopenia
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58
Q

What is a feared complication of aplastic anemia?

A

Transformation into an acute leukemia

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59
Q

How do you make the diagnosis of aplastic anemia?

A
  1. ) The peripheral smear will show a normochromic normocytic anemia.
  2. ) Bone marrow biopsy will show hypocellular marrow and the absence of progenitor cells for all 3 cell lines.
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60
Q

What are the two reactions in which B12 is an important cofactor?

A
  1. ) Homocysteine to Methionine

2. ) Methylmalonyl CoA to Succinyl CoA.

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61
Q

Where is vitamin B12 absorbed?

A

In the terminal ileum while bound to intrinsic factor (produced by gastric parietal cells).

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62
Q

What are the causes of vitamin B12 deficiency?

A
  1. ) Pernicious anemia
  2. ) Gastrectomy
  3. ) Poor diet (vegetarianism, alcoholism)
  4. ) Crohn’s Disease, Ileal resection
  5. ) Organisms competingg for B12–> Diphyllobothrium latum (fish tapeworm), blind loop syndrome (bacterial overgrowth).
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63
Q

Define pernicious anemia

A

An autoimmune condition resulting in inadequate production of intrinsic factor by the gastric parietal cells. This leads to impaired absorption of vitamin B12 in the terminal ileum.

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64
Q

Clinical features of vitamin B12 deficiency:

A
  1. Anemia
  2. Sore tongue
  3. Neuropathy
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65
Q

What are the neurologic manifestations of vitamin B12 deficiency?

A
  1. ) Neuropathy secondary to demyelination of the posterior columns, the lateral CST< and the spinocerebellar tracts
  2. ) Impaired proprioception
  3. ) Ataxia
  4. ) UMN signs, hyperreflexia, spasticity, weakness, upgoing Babinski.
  5. ) Urinary and fecal incontinence
  6. ) Dementia
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66
Q

What are 2 things you would see on a peripheral smear to diagnose B12 deficiency?

A
  1. ) Macrocytic RBCs (MCV >100)

2. ) Hypersegmented neutrophils

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67
Q

What two values in the serum will be elevated with vitamin B12 deficiency?

A
  1. ) Elevated methylmalonic acid

2. ) Elevated homocysteine levels

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68
Q

What is the treatment for vitamin B12 deficiency?

A

Cyanocobalamin (vitamin b12) IM once per month.

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69
Q

How long does it take to become deficiency in folate? in B12?

A

Folate–> 3 months

B12–> 3+ years

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70
Q

What are the causes of folate deficiency?

A
  1. ) Inadequate dietary intake “tea and toast” diet
  2. ) Alcoholism
  3. ) Long term use of antibiotics
  4. ) Increased demand
  5. ) Pregnancy
  6. ) hemolysis
  7. ) Use of folate antagonists like methotrexate
  8. ) Anticonvulsant medications (phenytoin)
  9. ) Hemodialysis
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71
Q

What is the treatment for folate deficiency?

A

Daily oral folic acid replacement

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72
Q

What are the serum levels of methylmalonic acid and homocysteine in folate deficiency compared to B12 deficiency.

A

In folate deficiency, serum levels of homocysteine are high but methylmalonic acid levels are normal. Therefore if both are elevated, it must be a vitamin B12 deficiency.

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73
Q

Hemolysis due to external RBC defects can be caused by?

A
  1. ) Immune hemolysis
  2. ) Mechanical hemolysis (prosthetic heart valves, MAH)
  3. ) Medications, burns, toxins

Most of these are ACQUIRED cases of hemolysis.

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74
Q

Hemolysis due to internal RBC defects can be caused by?

A
  1. ) Hemoglobin abnormality like sickle cell dz, HbC dz, thalassemia
  2. ) Membrane defects like hereditary spherocytosis, PNH
  3. ) Enzyme defects like G6PD deficiency, pyruvate kinase deficiency
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75
Q

Where does intravascular hemolysis occur? extravascular?

A

Intravascular–> within the circulation

Extravascular–> within the reticuloendothelial circulation, the spleen.

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76
Q

What are the clinical features of hemolytic anemias?

A
  1. Signs and symptoms of anemia
  2. Jaundice
  3. Signs and symptoms of whatever underlying disease they have
  4. Dark color of urine (due to hemoglobinuria)
  5. Hepatosplenomegaly, cholelithiasis, lymphadenopathy
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77
Q

Does hemoglobinuria indicate intra or extravascular hemolysis?

A

Intravascular hemolysis.

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78
Q

What are the typical lab values/findings seen in hemolytic anemia?

A
  1. ) Anemia
  2. ) Elevated retic count
  3. ) Elevated LDH
  4. ) Decreased haptoglobin
  5. ) Decreased hemoglobin and hematocrit
  6. ) Elevated indirect bilirubin
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79
Q

G6PD Deficiency peripheral smear

A

Heinz Bodies

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80
Q

Extravascular hemolysis on peripheral smear

A

Helmet cells or spherocytes

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81
Q

Intravascular hemolysis on peripheral smear

A

Schistocytes

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82
Q

Why is haptoglobin low in hemolytic anemias?

A

Haptoglobin binds to hemoglobin so when haptoglobin is low it indicates that hemoglobin has been destroyed.

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83
Q

What is the specific mutation that causes sickle cell disease?

A

Uncharged valine is substituted for a negatively charged glutamic acid at the sixth position of the beta-chain.

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84
Q

What type of conditions cause sickling?

A

Decreased oxygen conditions: acidosis, hypoxia, changes in temperature, dehydration, and infection.

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85
Q

Patients with sickle cell trait often have this unique finding in their urine:

A

Isothenuria. This is the inability to concentrate or dilute the urine. Patients have a constant osmolality on urine testing.

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86
Q

What causes hand foot syndrome/dactylitis in SCD?

A

Avascular necrosis of the metacarpal and metatarsal bones. This is often the first manifestation of sickle cell disease. Causes painful swelling of the dorsa of the hands and feet seen in infancy and early childhood.

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87
Q

What are 3 medical interventions that can be done to prevent complications of sickle cell disease.

A
  1. ) Early vaccination against S. Pneumo, H. Flu, and N. Mening
  2. ) Prophylactic PCN from 4 mos to 6 yrs old.
  3. ) Folate supplementation due to chronic hemolysis
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88
Q

Hereditary Spherocytosis:

  • Inheritance pattern
  • Pathology
A
  1. Autosomal dominant
  2. Defect in the gene coding for spectrin and other RBC proteins. There is a loss in RBC surface area without a reduction in RBC volume, necessitating a spherical shape. The spherical RBCs become trapped and destroyed in the spleen by macrophages–> extravascular hemolysis.
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89
Q

What are the causes of spherocytosis? There are 5.

A
  1. Hereditary spherocytosis
  2. G6PD deficiency
  3. Hyperthermia
  4. ABO incompatibility (but not Rh incompatibility)
  5. Autoimmune hemolytic anemia
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90
Q

What are the clinical features of hereditary spherocytosis?

A
  • Hemolytic anemia
  • Jaundice
  • Splenomegaly
  • Gallstones
  • Occasional hemolytic crises
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91
Q

What is the treatment of choice for hereditary spherocytosis?

A

Splenectomy

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92
Q

Describe the osmotic fragility test as it relates to hereditary spherocytosis.

A

The osmotic fragility test tests the ability of RBCs to swell in a graded series of hypotonic solutions. Because of their shape, spherocytes tolerate less swelling before they rupture; thus they are osmotically fragile. The RBCs undergo lysis at a higher/earlier oncotic pressure.

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93
Q

G6PD Deficiency:

  • Inheritance pattern
  • Pathology
A
  1. X-linked recessive

2. defect in glucose-6-phosphate dehydrogenase that results in hemolytic episodes caused by precipitating factors.

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94
Q

Known precipitants to G6PD:

A
  • fava beans
  • sulfonamides
  • nitrofurantoin
  • primaquine (anti-malarials)
  • dimercaprol
  • infection
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95
Q

What are the clinical features of G6PD deficiency?

A
  • Episodic hemolysis

- Jaundice and dark urine on examination

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96
Q

What does a peripheral blood smear show in a patient with G6PD?

A
  • Bite Cells

- Heinz Bodies

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97
Q

What are bite cells

A

Bite Cells are RBCs after the removal of Heinz bodies by phagocytes like splenic macrophages.

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98
Q

What are Heinz bodies?

A

Heinz bodies are abnormal hemoglobin precipitates within the RBCs.

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99
Q

How do you treat G6PD?

A
  • Transfuse as necessary
  • Avoid precipitants of hemolysis
  • Maintain hydration
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100
Q

Define the pathology of AIHA (autoimmune hemolytic anemia).

A

This is caused by the production of autoantibodies that target RBC membrane antigens, leading to the destruction of these RBCs.

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101
Q

Which autoantibody class is produced in WARM AIHA?

A

IgG

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102
Q

What is the pathology of Warm AIHA?

A

IgG autoantibodies bine to RBC membranes optimally at 37 degrees C. This results in EXTRAVASCULAR hemolysis therefore Splenomegaly is a common feature.

It’s WARM OUTside

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103
Q

What are some causes of warm AIHA?

A
  • Idiopathic
  • SLE
  • Alpha methyldopa
  • leukemias, lymphomas
  • Collagen vascular disease
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104
Q

What autoantibody is produced in COLD AIHA?

A

IgM

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105
Q

What is the pathology of Cold AIHA?

A

IgM antibodies are produced and bind optimally to the RBC membranes at cold temperatures like 0 degrees celsius. This produces complement activation and results in intravascular hemolysis. The primary site of RBC sequestration in cold AIHA is the liver.

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106
Q

What are some causes of Cold AIHA?

A
  • Mycoplasma Pneumonia
  • Mononucleosis
  • Idiopathic
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107
Q

In a direct coombs test, if the RBCs are coated with IgG, what is the diagnosis?

A

The diagnosis would be Warm AIHA. This is a positive direct coombs test. Spherocytes may be present in Warm AIHA.

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108
Q

In a direct coombs test, if the RBCs are only coated with complement, what is the diagnosis?

A

The diagnosis would be Cold AIHA. Also, if there is a positive cold agglutinin titer, then the diagnosis is cold AIHA.

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109
Q

Treatment of Warm AIHA

A
  • Glucocorticoids
  • Splenectomy if non-responsive to glucocorticoids
  • Immunosuppression with azathioprine or cyclophosphamide
  • RBC transfusions if absolutely necessary
  • Folic acid supplements
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110
Q

Treatment of Cold AIHA

A
  • Avoid cold exposure
  • RBC transfusions if absolutely necessary
  • Various chemotherapeutic agents
  • Steroids are NOT helpful for COLD AIHA
111
Q

What is the pathophysiology of paroxysmal noctural hemoglobinuria?

A

This is caused by a deficiency of anchor proteins that link complement-inactivating proteins to blood cell membranes. The deficiency of this anchoring mechanism results in an unusual susceptibility to complement mediated lysis of RBCs, WBCs, and platelets.

112
Q

What are the clinical features of PNH?

A
  1. Chronic intravascular hemolysis
  2. Normochromic normocytic anemia
  3. Pancytopenia
  4. Thrombosis of venous system (look for Budd Chiari)
  5. Abdominal, back, and MSK pain
  6. May evolve into aplastic anemia, myelodysplasia, myelofibrosis, and acute leukemia.
113
Q

What 3 tests can you do to diagnose PNH?

A
  1. Ham Test
  2. Sugar water test
  3. Flow cytometry looking for CD59 and CD55.
114
Q

Describe the Ham Test

A

The patient’s cells are incubated in acidified serum, triggering the alternative complement pathway, resulting in lysis of PNH cells but not normal cells.

115
Q

What is the treatment for PNH?

A
  1. Glucocorticoids like prednisone. Many patients will not respond.
  2. Bone marrow transplant.
116
Q

Heparin Induced Thrombocytopenia Type 1

A

Heparin directly causes platelet aggregation. Seen <48 hours after initiating heparin. No treatment is needed.

117
Q

Heparin Induced Thrombocytopenia Type 2

A

Heparin induces antibody-mediated injury to platelets; seen 3-12 days after initiating heparin; heparin should be discontinued immediately.

118
Q

Describe the pathology of ITP

A

Immune (Idiopathic) Thrombocytopenic Purpura: This results from autoimmune antibody formation against host platelets. These antiplatelet antibodies are IgG antibodies that coat and damage the platelets which are then removed by splenic macrophages.

119
Q

Acute Form of ITP

A
  • occurs in children
  • follows a viral infection
  • self-limited
120
Q

Chronic Form of ITP

A
  • occurs in adults, women of childbearing age

- spontaneous remission is rare.

121
Q

At what platelet count do you get major spontaneous bleeding like intracranial bleeding or heavy GI bleeding?

A

<5000

122
Q

At what platelet count do you see minor spontaneous bleeding like easy bruising, petechiae, epistaxis, menorrhagia or bleeding gums?

A

<20,000

123
Q

Describe the clinical features of ITP

A
  • petechiae and ecchymosis on the skin
  • bleeding of mucus membranes
  • NO splenomegaly
124
Q

How do you diagnose ITP?

A
  • Platelet count <20,000
  • Blood smear normal aside from thrombocytopenia
  • Bone marrow aspirate shows increased megakaryocytes
  • Increased amount of platelet IgG
125
Q

How do you treat ITP?

A
  1. Adrenal corticosteroids
  2. IVIG
  3. Splenectomy (chronic ITP)
  4. Romiplostim and Eltrombopag–> both are drugs that work as thrombopoietin receptor agonists to increase platelet production.
126
Q

Describe the pathology of TTP.

A

Thrombotic Thrombocytopenic Purpura: a rare disorder of platelet consumption. Patient with TTP lack a functional ADAMTS13, which is a protease that cleaves vWF. Ultralarge vWF multimers buildup in the blood as a result of this deficiency. Microthrombi (mostly platelet thrombi) occlude small vessels leading to microangiopathic hemolytic anemia.

127
Q

How are PT/PTT changed with TTP?

A

PT and PTT are normal in TTP because there is no consumption of clotting factors.

128
Q

Compare TTP and HUS.

A

TTP = HUS + fever + AMS

129
Q

Describe the clinical features of TTP:

A 
FAT RN: 
Fever 
Anemia (microangiopathic hemolytic) 
Thrombocytopenia 
Renal failure (acute/mild) 
Neurologic changes (fluctuating)
130
Q

What is the treatment for TTP?

A
  1. Plasmapheresis

2. Corticosteroids and splenectomy

131
Q

Should you give platelets for TTP?

A

NO

132
Q

In HIT, what are the antibodies formed against?

A

Autoantibodies are formed against heparin-platelet factor 4 complex.

133
Q

Which type of heparin has a much lower risk of HIT?

A

LMWH

134
Q

What is the feared complication of HIT?

A

A DVT or PE

135
Q

A decrease in platelet count by what percentage indicates the possibility of HIT?

A

If platelets drop to 50% of initial value, consider HIT

136
Q

How do you diagnose HIT?

A
  1. Antiplatelet factor IV antibody

2. Serotonin release assay

137
Q

How do you treat HIT?

A
  1. STOP Heparin
  2. give thrombin inhibitor such as Dabigatran, Argatroban, or Lepirudin.
  3. Avoid heparin in the future in any patient that developed HIT.
138
Q

Bernard-Soulier Syndrome:

  • Inheritance Pattern
  • Pathology
A
  1. Autosomal Recessive Disease

2. Disorder of platelet adhesion to the subendothelium due to a deficiency of thee platelet glycoprotein GpIb-IX

139
Q

What is seen in lab/smear in Bernard-Soulier Syndrome?

A
  • Platelet count is low

- Platelets are abnormally large

140
Q

Glanzmann Thrombasthenia:

  • Inheritance pattern
  • Pathology
A
  • Autosomal Recessive Disease

- Disorder of platelet aggregation due to deficiency in platelet glycoprotein GPIIb-IIIa

141
Q

What is seen in lab/smear in Glanzmann Thrombasthenia?

A
  • Bleeding time is prolonged

- Platelet count is normal

142
Q

Reversible causes of sideroblastic anemia

A

Lead, EtOH, INH

143
Q

Irreversible causes of sideroblastic anemia

A

Metabolic disease of B6, AML/Myelodysplasia

144
Q

How do you confirm the diagnosis of G6PD?

A

Get a G6PD level 6-8 weeks after the attack. Levels will be normal during the attack because young cells have G6PD.

145
Q

What is the best test for diagnosing hereditary spherocytosis?

A

Osmotic fragility

146
Q

Inheritance of vWD

A

autosomal dominant

147
Q

What is the role of vWF?

A

vWF enhanced platelet aggregation and adhesion by binding to Gp1B-IX on platelets. It also acts as a carrier of factor VIII in the blood.

148
Q

Where is vWF made?

A

the endothelial cells and megakaryocytes.

149
Q

How do you diagnose vWF?

A

Prolonged bleeding time, normal platelet count, PTT may or may not be prolonged, decreased plasma vWF, decreased factor VIII, reduced ristocetin induced platelet aggregation.

150
Q

What medication can you give for vWD and how does it work?

A

DDAVP, it induces endothelial cells to secrete vWF.

151
Q

Why should you not give cryoprecipitate for vWD?

A

Because of the risk of viral transmission.

152
Q

What is the inheritance pattern of Hemophilia A?

A

X-linked Recessive

153
Q

What clinical features are unique to hemophilia A or factor abnormalities as compared to platelet abnormalities?

A

Hemarthroses which can lead to progressive joint destruction.

154
Q

What value is prolonged in Hemophilia A?

A

PTT

155
Q

How do you treat hemarthroses associated with Hemophilia A?

A

Analgesics (not aspirin or NSAIDs), joint immobilization, ice packs, nonweight bearing.

Treat Hemophilia A in general with factor VIII replacement.

156
Q

PT

A

represents the extrinsic pathway, prolonged by warfarin

157
Q

PTT

A

represents the intrinsic pathway, prolonged by heparin

158
Q

Thrombin Time

A

Measurement of the fibrinogen concentration

159
Q

Bleeding Time

A

Reflects platelet function

160
Q

Normal PT

A

11-15 seconds

161
Q

Normal PTT

A

25-40 seconds

162
Q

Normal bleeding time

A

2-7 minutes

163
Q

What is DIC?

A

Abnormal activation of the clotting system, leading to the formation of microthrombi throughout the microcirculation. This causes consumption of platelets, fibrinogen, and coagulation factors. Fibrinolytic mechanisms are activated thus leading to hemorrhage. Therefore, bleeding and thromboses are occurring simultaneously.

164
Q

List some causes of DIC

A
  • Infection
  • Malignancy
  • Pregnancy (placental abruption, amniotic fluid emboli, retained POC)
  • Major Tissue Injury from trauma or burns
  • APL
  • Shock
  • Snake Venom
165
Q

What levels are elevated in DIC?

A

PT, PTT, Bleeding Time, Fibrin split products, D-dimer

166
Q

What levels are decreased in DIC?

A

Platelets, fibrinogen

167
Q

What is seen on a peripheral smear in DIC?

A

Schistocytes

168
Q

Tx of DIC?

A

Supportive, O2, IVF, BP support, treat the underlying cause

169
Q

What factors depend on vitamin K for synthesis?

A

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

170
Q

What are some complications of DIC?

A

Intracranial bleeding, stroke, PE, bowel infarction, acute renal failure

171
Q

What are some causes of Vitamin K Deficiency?

A
  • Prolonged Abx Use and NPO
  • Patients on TPN
  • Malabsorption of fat soluble vitamins
  • Warfarin
172
Q

Which factor has the shortest half life?

A

VII

173
Q

What lab value will be the first to be prolonged after initiation of warfarin?

A

PT because VII has the shortest half life.

174
Q

What are the 3 reasons why coagulopathy develops in liver disease?

A
  1. Clotting factors are made in the liver, so there is decreased synthesis
  2. Cholestasis leads to decreased absorption of vitamin K
  3. Hypersplenism/splenomegaly due to portal hypertension causes thrombocytopenia.
175
Q

What are some treatment options for liver coagulopathy?

A
  • FFP
  • Platelet transfusion
  • Vitamin K
  • Cryoprecipitate
176
Q

Antithrombin III Deficiency inheritance pattern

A

autosomal dominant

177
Q

What is the MOA of AT3?

A

Antithrombin III inhibits thrombin. Therefore, when there is a deficiency of AT3, thrombin has free reign and keeps on making clots.

178
Q

What are some clinical features of antiphospholipid antibody syndrome?

A
  • Recurrent fetal loss
  • arterial or venous thromboembolism
  • thrombocytopenia
179
Q

Protein C Deficiency Inheritance Pattern

A

Autosomal dominant

180
Q

what is the MOA of protein C?

A

Protein C inhibits factor V and VIII which are both part of the clotting cascade. Therefore when protein C is deficient, clots continue to form.

181
Q

What is the MOA of protein S/protein S deficiency?

A

Protein S is a cofactor for protein C. When protein S is deficient, protein C can’t adequately inhibit factor V and VIII.

182
Q

What is the MOA of factor V leiden mutation?

A

This leads to a mutation in factor V which results in the inability of protein C to inactivate factor V therefore there is continuous clotting.

183
Q

In what clinical scenarios should you consider that someone has an inherited hypercoagulable disorder?

A
  • Recurrent thromboses
  • Family hx of recurrent thromboses
  • First thrombotic event occurs before the age of 40.
  • Patient experiences thromboses in unusual sites.
184
Q

When should someone be permanently anticoagulated? (in reference to hypercoagulable disorders)

A

When they have had 2 or more thrombotic events.

185
Q

What is the MOA of heparin?

A

Heparin potentiates the action of antithrombin III to inhibit factor IIa and Xa

186
Q

What are some adverse effects of heparin?

A
  • Bleeding
  • HIT
  • Alopecia
  • Osteoporosis
  • Rebound hypercoagulability after stopping drug
187
Q

What are the contraindications to heparin

A
  • Active bleeding, intracranial or GI
  • Recent surgery on spine, brain, or eyes
  • Severe HTN
  • Previous HIT
  • Hemophilia or thrombocytopenia
188
Q

What is the MOA of LMWH

A

LMWH mostly inhibits factor Xa but has less inhibition of factor IIa and platelet aggregation.

189
Q

What are the drugs that are LMWH

A

enoxaparin, dalteparin, and tinzaparin

190
Q

What is the benefit of LMWH?

A

Less side effects, given SC, don’t have to monitor PTT

191
Q

How is LMWH excreted and in which population should you use caution?

A

Renally excreted, use caution in those with renal failure

192
Q

What is the MOA of warfarin?

A

Vitamin K antagonist

193
Q

How long does it take for the anticoagulant effect of warfarin to occur?

A

4-5 days–> bridge w/ heparin

194
Q

What is the target INR for people with mechanical heart valves?

A

2.5-3.5

195
Q

Adverse effects of warfarin?

A
  • Hemorrhage
  • Skin Necrosis
  • Teratogenic
  • DON’T GIVE TO PEOPLE AT RISK FOR FALLS (elderly, alcoholics)
196
Q

Why does skin necrosis occur with warfarin?

A

It is caused by a rapid decrease in protein C and S, (which actually increases clotting).

197
Q

What is the MOA of clopidogrel

A

It blocks the binding of ADP to the PY12 receptor which reduced platelet activation and aggregation.

198
Q

What are the direct factor Xa inhibitors?

A

Rivaroxaban, Apixaban, Edoxaban

199
Q

What are the direct thrombin (IIa) inhibitors?

A

Dabigatran, argatroban, lepirudin

200
Q

Define monoclonal gammopathy of undetermine significance

A

It is an asymptomatic premalignant clonal plasma cell proliferation.

201
Q

How do you treat MGUS?

A

Don’t do anything, just closely observe them to monitor for transformation to multiple myeloma.

202
Q

How do you make the diagnosis of MGUS?

A
  • IgG spike
  • Bence-Jones Proteinuria
  • NO end organ damage
203
Q

Define multiple myeloma

A

A neoplastic proliferation of a single plasma cell line that produces monoclonal immunoglobulin.

204
Q

Define CRAB in terms of multiple myeloma

A

C-Hypercalcemia
R-renal failure
A-anemia
B-bone lesions (lytic)

205
Q

List 5 clinical features of Multiple Myeloma

A
  1. ) Skeletal manifestations
  2. ) Increased risk of infection
  3. ) Renal failure
  4. ) Anemia
  5. ) Cord compression
206
Q

How does renal failure develop in Multiple Myeloma?

A

Myeloma Nephrosis-immunoglobulin precipitation in the renal tubules leads to tubular casts of Bence Jones proteins.
Also hypercalcemia contributes to the renal decompensation.

207
Q

Why are patients with multiple myeloma at risk for recurrent infections?

A

because they are only producing one type of immunoglobulin and there is deprivation of normal immunoglobulins therefore humoral immunity is compromised.

208
Q

How does cord compression occur in multiple myeloma?

A
  1. Plasmacytoma

2. Fracture from bone fragment

209
Q

What are some lab findings of multiple melanoma?

A
  • Hypercalcemia
  • Elevated ESR
  • Rouleaux formation
  • Increased total protein
  • Bence Jones protein in urine
  • Elevated Creatinine
  • Leukopenia, thrombocytopenia, and anemia.
210
Q

How do you make the diagnosis of multiple melanoma?

A
  • Serum and urine protein electrophoresis will show M-protein (monoclonal protein)
  • Bone scan to detect lytic lesions
  • Bone marrow biopsy
211
Q

What causes the osteolytic lesions of multiple myeloma?

A

Osteolytic lesions are secondary to the release of osteoclast-activating factor by the neoplastic plasma cells

212
Q

What is the preferred treatment for multiple myeloma?

A

Hematopoietic cell transplantation. Usually reserved for younger and relatively asymptomatic patients.

213
Q

What are other treatment options for multiple myeloma?

A
  • Chemotherapy –> alkylating agents

- Radiation therapy

214
Q

What is Waldenstrom Macroglobulinemia

A

A malignant proliferation of plasmacytoid lylmphocytes. These lymphocytes produce IgM paraprotein which causes a hyperviscosity syndrome.

215
Q

Are there bone lesions in Waldenstrom Macroglobulinemia?

A

NO

216
Q

What are the clinical features of Waldenstrom Macroglobulinemia?

A

Fatigue, weight loss, neurologic symptoms, lymphadenopathy, splenomegaly, anemia, abnormal bleeding, hyperviscosity syndrome which can cause retinal vessel dilation with hemorrhage and blindness.

217
Q

What mutation is commonly found in polycythemia vera?

A

JAK2 kinase

218
Q

What are symptoms of hyperviscosity associated with PV?

A

Headache, dizziness, weakness, pruritus (esp. after taking a shower), visual impairment, dyspnea

219
Q

What other clinical manifestations in addition to hyperviscosity can be seen with PV?

A
  • Thrombotic phenomenon: DVT, CVA, MI, portal vein thrombosis
  • Bleeding
  • Splenomegaly, hepatomegaly
  • HTN
220
Q

What are some secondary causes of polycythemia?

A

CO poisoning, hypoxemia

221
Q

What lab values are seen with PV? Hgb, Hct, leuks etc.

A 
RBC-elevated 
Hgb-elevated 
Hct-elevated (often >50) 
Thrombocytosis 
Leukocytosis 
Decreased serum levels of EPO 
Elevated B12 
Hyperuricemia
222
Q

How do you confirm the diagnosis of polycythemia vera?

A

Bone marrow biopsy

223
Q

Treatment for PV?

A

Repeated phlebotomy to lower the Hct

224
Q

How do you make the diagnosis of a myelodysplastic syndrome?

A
  • Normal or mildly elevated MCV
  • Low retic count (BM isn’t able to respond!)
  • Howell Jolly Bodies
  • Basophilic stippling
  • Nucleated RBCs
  • Large agranular platelets
225
Q

Treatment of myelodysplastic syndrome

A
  • RBC and platelet transfusion
  • EPO
  • Granulocyte CSF
  • Vitamin Supplementation (B6, B12, Folate)
  • BMT is the only potential cure
  • Chemo, immunosuppressive agents have variable results
226
Q

Define essential thrombocytosis

A

Platelet count >600,000

227
Q

How does essential thrombocytosis usually manifest?

A

As a thrombotic event such as CVA. Could also present with paradoxical bleeding due to defective platelet function.

228
Q

What are some other clinical findings seen with ET?

A

Splenomegaly
Pseudohyperkalemia
Elevated bleeding time
Erythromelalgia (burning pain and erythema of the extremities due to microvascular occlusions.

229
Q

What will a peripheral smear in ET show?

A

Hypogranular abnormally shaped platelets

230
Q

What will a bone marrow biopsy show in ET?

A

An increased number of megakaryocytes

231
Q

Treatment of ET?

A
  • Antiplatelet agents like: Anagrelide, low dose aspirin

- Hydroxyurea

232
Q

Which type of lymphoma has the worst prognosis?

A

Lymphocyte depleted type

233
Q

What are the four subtypes of Hodgkin lymphoma?

A
  • Nodular sclerosing
  • Lymphocyte predominant
  • Mixed Cellularity
  • Lymphocyte Depleted
234
Q

What cell is associated with HL?

A

Reen Sternberg

235
Q

Describe the Ann Arbor Staging System

A

Stage 1: Confined to single lymph node
Stage 2: involvement of >/= 2 lymph nodes but on the same side of the diaphragm
Stage 3: Both sides of the diaphragm involved
Stage 4: metastatic disease to extralymphatic sites

236
Q

Subtypes of HL-define A and B

A

A-no B symptoms

B-B symptoms present (fever, weight loss, night sweats)

237
Q

What is the most common symptom of Hodgkin Lymphoma

A

Painless lymphadenopathy

238
Q

How does Hodgkin Lymphoma Spread?

A

Anatomically, from one lymph node to the next

239
Q

What are 2 unique clinical signs associated with HL?

A
  • Pel Epstein Fevers

- Etoh lymphadenopathy

240
Q

What 2 chemo regimens are used for HL?

A

BEACOPP and ABVD

241
Q

How do you make the diagnosis of HL? There are 4 things

A
  1. Lymph node biopsy w/ the presence of Reed-Sternberg Cells
  2. Presence of inflammatory cell infiltrates (distinguishes HL from NHL, these are reactive to the Reed-Sternberg cells)
  3. CXR and CT scan to determine extent of involvement
  4. Bone marrow biopsy to evaluate bone marrow involvement
242
Q

Stages I, II, and IIIa of HL can be treated with?

A

Radiotherapy alone to the involved field.

243
Q

Stages IIIb and IV of HL are treated with?

A

Chemotherapy

244
Q

How does NHL spread?

A

Non-contiguously to blood and bone marrow.

245
Q

Where can a primary tumor in NHL be found?

A

GI tract

246
Q

Which disease is more common? HL and NHL and who usually presents with more advanced disease?

A

NHL is twice as common as HL. NHL typically presents with more advanced disease, stage IIb or greater.

247
Q

What are some risk factors for NHL?

A
  • Immunosuppression
  • HIV/AIDs
  • EBV, HTLV-1 viral infections
  • H. Pylori infection (increases risk of gastric associated lymphoma)
  • Autoimmune disease like Sjogrens or Hashimotos that increases risk of mucosa associated lymphoid tissue (MALT).
248
Q

What are some of the presenting signs of NHL?

A
  • Lymphadenopathy (painless, firm, and mobile)
  • HSM and early satiety/fullness
  • B Symptoms (less common than in HL)
  • Recurrent infections, symptoms of anemia or thrombocytopenia due to bone marrow involvement
  • other findings include SVC syndrome, bone pain, respiratory involvement, skin lesions etc.
249
Q

How do you diagnose HL?

A
  • LYMPH NODE BIOPSY
  • Other tests that may help: CXR and CT to determine extent of spread, serum LDH and beta-2 microglobulin are indicators of tumor burden, if alk phos is elevated, liver or bone involvement is likely. If LFTs or Bili is elevated liver involvement is likely; CBC, serum electrolytes, renal function tests, bone marrow biopsy
250
Q

What is the diagnostic criteria or guideline for biopsy a lymph node?

A

ANY LYMPH NODE >1 CM PRESENT FOR MORE THAN 4 WEEKS THAT CANNOT BE ATTRIBUTED TO INFECTION SHOULD BE BIOPSIED

251
Q

What is the chemo regimen for NHL?

A

R-CHOP

Rituxumab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone

252
Q

Risk factors for AML?

A

Exposure to radiation, myeloproliferative syndromes, down syndrome and chemotherapy

253
Q

Translocation for APML?

A

t(15;17)

254
Q

Treatment for APML

A

ATRA

255
Q

Complications of APML

A

DIC 2/2 Auer Rods

256
Q

What is the most common malignancy in children under age 15 in the US?

A

ALL-neoplasm of early lymphocytic precursors.

257
Q

What is the feared complication of ALL and what do you do about it?

A

CNS involvement, prophylactically treat w/ intrathecal chemo

258
Q

Which leukemia involves the testicles?

A

ALL

259
Q

Which leukemia involves a mediastinal mass?

A

T-cell ALL

260
Q

Which leukemia involves skin nodules?

A

AML

261
Q

Lab abnormalities seen in tumor lysis syndrome?

A 
Hypocalcemia 
Hyperphosphatemia 
Hyperkalemia 
Hyperuricemia 
This is a medical emergency!
262
Q

What is the most common leukemia after age 50?

A

CLL

263
Q

Is CLL scary?

A

No. They are more likely to die with it than from it.

264
Q

How does CLL present?

A

Usually asymptomatic but picked up on a routine CBC as lymphocytosis. May have generalized painless lymphadenoptahy, splenomegaly, infections etc.

265
Q

What is the CBC leukocyte range in CLL?

A

50k-200k. HIGH

266
Q

What is the classic cell seen on a peripheral smear in CLL?

A

Smudge cell. These are fragile leukemic cells that are broken when placed on a glass slide.

267
Q

Is CLL lymphocytic or myelogenous?

A

It is a monoclonal proliferation of lymphocytes that are morphologically mature but functionally defective meaning they do not differentiate into antibody manufacturing plasma cells.

268
Q

How do you differentiate CML from a Leukemoid reaction?

A

In a leukemoid reaction:

  • Usually no splenomegaly
  • Leukocyte alkaline phosphatase is HIGH
  • precipitating event like infection
269
Q

That is the pathogenesis of CML?

A

Translocation between t(9:22) resulting in BCR-ABL constituitively active tyrosine kinase that result in neoplastic clonal proliferation of myeloid stem cells.

270
Q

What is the feared complication of CML?

A

Transformation to an acute phase or blast crisis

271
Q

What do we treat CML with?

A

TKIs: Imatinib, Dasatinib, or Nilotinib.

272
Q

What are some of the presenting signs of CML?

A

fevers, night sweats, fatigue, weight loss, recurrent infections, easy bruising/bleeding, anemia symptoms.
splenomegaly, hepatomegaly, LAD.

273
Q

What lab findings allow you to make the diagnosis of CML?

A
  • LOW LAP
  • Marked leukocytosis
  • small number of blasts
  • leukemic cells in the peripheral blood
  • Thrombocytosis
  • Bone marrow biopsy will show leukemic cells.

Internal Medicine (6 decks)

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