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General Medicine > Hematology - Misc. Material + Broken Spreadsheets > Flashcards

Hematology - Misc. Material + Broken Spreadsheets Flashcards

1
Q

Common Hematology Complaints

(list 9)

A
  1. Fatigue
  2. Weakness
  3. Lightheadedness
  4. Pallor
  5. Shortness of breath
  6. Easy bruising
  7. Bleeding problems
  8. Frequent illness
  9. Palpitations
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2
Q

Common “Fatigue” Differentials

(list 5)

A
  1. Anemia
  2. Thyroid disorder
  3. Insufficient sleep
  4. Depression
  5. Drug use
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3
Q

Important Historical Questions in Eval Anemia

(6)

A
  1. Onset of s/sx, duration indicates chronic disease or acute stressor
  2. Nutrition, **especially vegan or vegetarian
  3. Sleep patterns
  4. Signs of bleeding
    • Gums
    • Rectal (bloody stool, assume colon cancer until proven otherwise)
  5. Prevoius episodes
  6. Associated symptoms
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4
Q

Significant Clinical Exam Findings for General Hematologic Disorders

(8 categories, 11 findings)

A
  1. General Impression
    • Toxic appearance
  2. Skin
    • Petichiae
    • Purpura
  3. HEENT
    • Conjunctiva/gums pale or spontaneously bleeding
    • Tongue c smooth surface
  4. Lymphatic
    • lymphadopathy
  5. Cardiac
    • tachycardia
  6. Abdo
    • splenomegaly
    • hepatomegaly
  7. MSK
    • bone pain, shaft > joint
  8. Neuro
    • sensory abnormalities, stocking glove
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5
Q

Reasons for total RBC mass decrease

(list 3)

A
  1. Decreased production
  2. Abnormal maturation
  3. Increased destruction
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6
Q

Decreased Hemoglobin and Hematocrit Values

(male and female, just list lower borders)

A

Males
•Hgb < 13.5 g/dl
•Hct < 41%

Females
•Hgb < 12 g/dl
•Hct < 37%

*Note: these are ranges from Current. Each lab will send you their own reference values to go off of *

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7
Q

Anemia Etiology

(2 categories, place microcytic and macrotcytic anemias in each category)

A
  1. Congenital
    • Thalasemia
    • Sickle cell disease
  2. Acquired
    • Dietary
      • Iron Def anemia
      • Vit B12 deficiency anemia
      • Folic Acid deficiency
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8
Q

Fill in the blanks

A

See picture below. These are just supplimentary notes

Production defect – will look different depending on cause. Normally shaped RBC but insufficient amount

•Underlying renal or hepatic disease (EPO deficiency)
•Underlying bone marrow disease (cannot actually produce)
•Blood loss in this category; the RBCs will continue to produce at high levels but it is getting lost

Maturation defect – normal amount but not forming correctly

•Thalasemia is a hemoglobinopathy
•Nuclear – RNA and ribosomes are okay. Therefore they can produce cytoplasm as sluggish nucleus growth until the age of production

Survival Defect

•Cells are produced normally but are destroyed early
•Remember, malaria is a parasite
•Drugs can cause hemolysis

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9
Q

Most Common Anemia S/Sx

(5 symptoms, 6 signs)

A

Symptoms

  • Fatigue
  • Headache (decreased oxygen to brain)
  • Exertional dyspnea
  • Faintness
  • Cold extremities

Common signs

  • Tachycardia
  • Orthostatic hypotension (less blood, less pressure)
  • Pallor
  • Jaundice (hemolytic origin)
  • Smooth tongue (loss of papillae, nutrient deficiency origin)
  • Koilonychia (spoon shaped nails, iron deficiency origin)
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10
Q

Microcytic Anemia Disease Acronym

A

TICS

Thalasemias
Iron Deficiency Anemia
Anemia of Chronic Disease
Siderobastic Anemia

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11
Q

Define Iron Deficiency Anemia

A

Inadequate supply of iron for synthesis of hemoglobin

Most common cause of anemia worldwide, 20% of white female Americans have it

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12
Q

Etiology, Iron Deficiency Anemia

(4 general)

A

Iron absorption >1-1.5 mg/day (after absorption) due to:

  1. GI tract bleeding (acute or chronic)
    • Often colon cancer in elderly
    • Pt will usually present late in anemia disease progression
  2. Chronic asprin or NSAID use
    • Think geriatrics taking them for arthritis
  3. Low iron intake
  4. Increased Fe demands
    • Pregnancy/lactation
    • Heavy menstrual bleeding
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13
Q

Pathophysiology, Iron Deficiency Anemia

A

Heme molecule cannot bind oxygen without sufficient iron

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14
Q

S/Sx, Iron Deficiency Anemia

(6 signs, 5 symptoms)

A

Signs

  1. Brittle nails
  2. Koilonychia (spoon shaped nails)
  3. Pale conjunctiva
  4. Smooth tongue (typical of all malnutrition anemias)
  5. Tachycardia
  6. CHF, if untreated for prolonged period

Symptoms

  1. Fatigue
  2. Weakness
  3. Palpitations
  4. SOB
  5. Pica

Note - Pica is often a board question

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15
Q

Differential Diagnoses, Iron Deficiency Anemia

(3, c differntiating factors from iron deficiency)

A
  1. Anemia of chronic disease
    • Normal ferritin levels
    • Low Serum Fe
  2. Thallesemia
    • Sig. low MCV
    • Normal or inc. RBC
  3. Sideroblastic anemia
    • Inc. serum Fe
    • Inc. % saturation
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16
Q

Diagnostic Tests c Results, Iron Deficiency Anemia

(5 tests, listed in order in which they become abnormal in disease progression)

A
  1. CBC – microcytic hypochromic RBC in late anemia progression
  2. Serum ferritin – <12 mcg/dl is highly reliable (labs will provide norm. ranges)
  3. Serum Fe
  4. Total Iron Binding Capacity (TIBC)
  5. % Saturation (of Fe in proteins)
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17
Q

Medical Treatment, Iron Deficiency Anemia

(2)

A

Dependent on cause. Ultimately tx underlying reason

  1. Ferrous sulfate 325 mg po tid
    • Expect normal hematocrit within 3 weeks
    • Expect baseline within 2 mo
    • Continue 6 mo to restore Fe stores in liver and heart
    • *If pt resistant to pills, council on dietary habits *
  2. Parenteral Fe (only if oral fails)
    • Must be delivered in the hospital due to high risk for anaphylaxis
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18
Q

Patient Education/Prevention, Iron Deficiency Anemia

A

Once levels are returned to normal, practice etiology-specific practices to prevent recurrence

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19
Q

Normal Physiology of Iron Ingestion and Transport

A

Notes -

  1. only 10% of the 10-20 mg of dietary iron is absorbed
  2. transferrin carries all of the iron from the GI tract
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20
Q

Regulation of Iron

(4 general principles)

A
  • Iron absorption
  • Iron recycling via marcophages
  • Iron loss
  • Interaction c several transport PRO
  • Transferrin
    • Major transporter of Fe in palsma
  • Ferritin
    • Cellular storage of iron (most important testing is for this)
  • Ferroportin
  • Hepicidin
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21
Q

Serum Ferritin, Serum Fe, TIBC, % Saturation

(define and differentiate)

A

Serum Ferritin - Amount of stored iron bound to ferritin. This is iron from heart and liver. In iron deficiency anemia this will drop first as a compensatory mechanism

Serum Fe - Concentration of Fe bound to transferrin in plasma. This is the iron immediately availible for use in hemoglobin

TIBC, Total Iron Binding Capacity - Measurement of all proteins availible for Fe binding (empty seats on the bus)

% Saturation - Percentage of proteins saturated c Fe (full seats on the bus)

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22
Q

Define Anemia of Chronic Disease

A

Anemia from inflammatory disease → impaired marrow response to EPO

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23
Q

Etiology, Anemia of Chronic Disease

(4)

A

Associated c chronic diseases that chronically inflame

  • Rheumatoid arthritis
  • Chronic infection
  • Malignancy
  • Chronic renal disease (often comorbid c iron and folate deficiency anemias due to RBC loss from hemodialysis; look for ansiocytosis and polychromasia)
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24
Q

Pathophysiology, Anemia of Chronic Disease

A

Disruption originating from macrophages (still in research)

  • Macrophage release of cytokines (interleukins)
    • IL-6, IL-1β
    • Increased liver production of hepcidin
    • Hepcidin decreases iron absorption form GI epithelium
    • Hepcidin decreases recycled iron export from macrophages due to inhibited ferroportin
  • TNF-α, IL-1β
    • Inappropriate EPO production in kidney
    • Reduced erythropoiesis in BM

Bone marrow dysfunction due to various causes

  • T-cell mediated autoimmune suppression of hematopoiesis (most common)
  • Radiation therapy
  • Chemotherapy
  • Toxins
    • Chronic alcohol abuse
    • Lead
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25
Q

S/Sx, Anemia of Chronic Disease

(2 signs, 3 symptoms)

A

Symptoms

  1. Fatigue
  2. Weakness
  3. Symptoms consistent c underlying cause

Signs

  1. Pallor
  2. Signs consistent c underlying cause
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26
Q

Differential Diagnoses, Anemia of Chronic Disease

(3)

A

Other microcytic anemias

  1. Iron deficiency anemia
  2. Thallesemia
  3. Sideroblastic anemia

Differentiation b/w ACD and Fe Deficiency is often on the boards

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27
Q

Diagnostic Tests, Anemia of Chronic Disease

(4, order dependent on hx)

A
  1. CBC – normochromic normocytic anemia or microcytic, hypochromic anemia (90% of time microcytic)
  2. Serum Fe – decreased, iron sequestered in macrophages
  3. TIBC – decreased
  4. Serum ferritin – normal or increased, adequate iron in body but hepcidin prevents uptake. This is the key to differentiating ACD from Fe Deficiency
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28
Q

Medical Tx, Anemia of Chronic Disease

(3 c 1 general plan, cause dependent)

A

Generally, treat underlying disease and admin EPO injections

  1. Chronic Renal Insufficiency – hemodialysis + EPO (usually admin. together)
  2. Malignancy – chemo, radiation, EPO
  3. Inflammation (RA) – NSAIDs, DMARDs, EPO
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29
Q

Hepcidin

(Origin, 2 Functions)

A

Origin: produced in liver

Functions:

  1. Decrease iron absorption from GI tract after diffusion thru epithelial cells
  2. Decrease iron release from marcophages via pheroportin protein blockage

Plays important role in anemia of chronic disease

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30
Q

DMARD

(what it stands for, function, 3 examples)

A

Disease Modifying Antirheumatic Drugs

  • Function - *Used in combo c EPO to treat RA
  • Examples*
  1. Methotrexate
  2. Etanercept (embrel)
  3. Infliximab (remicade)
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31
Q

Define Thalasemia

A

Group of hereditary disorders characterized by reduction in synthesis of globin chains (α or β)

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32
Q

Etiology, Thalasemias

(2)

A
  • α thal – HBA1 and HBA 2 gene deletion causing reduced α-globin chain synthesis. Severity depends on how many present alleles out of 4
  • β thal – gene mutation terminating DNA chain responsible for β-globin prdctn. May be homozygous (major) or homozygous (minor)
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33
Q

Pathopysiology, Thalasemias

(3 steps)

A
  1. Reduced globin chain synthesis
  2. Reduced hemoglobin synthesis
  3. Hypochromatic mircocytic anemia
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34
Q

S/Sx, Thalasemias

(1 symptom, 5 alpha signs, 2 beta categories c 4 specifics)

A

Symptoms

Typical anemia symptoms c varying severity depending on etiology

Signs

  • α Thalasemia, severity depends on number of missing alleles
    • 4 alleles – normal hbg
    • 3 alleles – silent carrier, asymptomatic
    • 2 alleles – “trait” exhibits mild microcytic anemia symptoms
    • 1 allele – “Hbg H Disease” variable severity splenomegaly and pallor
    • 0 alleles – “hydrops fetalis” stillborn fetus
  • β Thalasemia
    • Major – symptom development upon unsuccessful Hbg F → Hbg A conversion
      • Growth failure
      • Bone deformities
      • Hepatosplenomegaly
      • Jaundice
    • Minor –clinically insignificant microcytic anemia (often discovered in routine physical
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35
Q

Differential Dx, Thalasemias

(1, c differentiating factors)

A

Iron deficient anemia, due to microcytic hypochromatic cells

  • Higher MCV
  • Abnormal Fe studies
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36
Q

Diagnostic Tests, Thalasemias

A
  1. CBC –
  • Microcytosis out of proportion to degree of anemia, RBCs also hypochromatic
    • MCV 55-70 fl
    • Poikilocytosis – abnml RBC shape
      • Target cells
  • Hgb between 3 and 6 gm/dL
  1. Serum Fe – normal
  2. Hemoglobin electrophoresis – definitive dx
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37
Q

Medical Tx, Thalasemias

(severity dependent, 3 txs)

A
  • Mild (α thal trait or β thal minor) – no tx
  • Hgb H disease – folic acid supplementation
  • β Thalasemia major
    • Chelation therapy
    • Transfusions
    • Folate supplementation

Note - iron supplimentation is contraindicated and dangerous for β Thal patients!

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38
Q

Surgical Tx, Thalasemias

(1)

A

Stem cell/bone marrow transplantation for β Thalasemia major

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39
Q

Pt Education/Prevention, Thalasemias

(3)

A
  • Highly inheritable, take a thorough hx
  • Patients usually live to 30’s until overcome c heart failure
  • Avg patient populations
    • α thal – SE Asian
    • β thal – Mediterranean (Greek, Sicilian)
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40
Q

Define Sideroblastic Anemia

A

Genetic or acquired defective heme synthesis

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41
Q

Etiology, Sideroblastic Anemia

(3 causes)

A
  1. Lead
  2. EtOH abuse
  3. Myelodysplasia (umbrella term)
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42
Q

Pathophysiology, Sideroblastic Anemia

A

Reduced Hgb synthesis due to inability to incorporate heme into protoporhyn. Results in iron accumulation in mitochondria.

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43
Q

S/Sx, Sideroblastic Anemia

(1 symptom, 1 sign)

A

Both consistent c general anemia, no identifying s/sx

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44
Q

Differential Dx, Sideroblastic Anemia

(3)

A

Other microcytic anemias

  1. Iron deficiency anemia
  2. Thallesemia
  3. Anemia of chronic disease
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45
Q

Diagnostic Tests, Sideroblastic Anemia

(4 c results)

A
  1. CBC
    • Normochromatic, micro AND macrocytic anemia
    • Basophilic stippling of RBC – rRNA remnants from impaired hgb synth
  2. Serum Fe – increased
  3. Lead test - positive in the presence of lead poisoning
  4. Bone marrow biopsy – diagnostic
    • Erythroid hyperplasia
    • Ringed sideroblasts (iron ring within nucleated RBC)
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46
Q

Medical Tx, Sideroblastic Anemia

(1)

A

Manage offending agent

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47
Q

Basophilic Stippling

A

Iron accumulations in mitochondria of RBC as a result of siderblastic anemia . Indicates sufficient iron levels but insufficient distribution to hemoglobin molecules

Note - Basophilic stippling occurs in conditions other than sideroblastic anemia. That is why a bone marrow biopsy is necessary for dx

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48
Q

Cooley’s Anemia

A

Just another name for Beta Thal Major

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49
Q

MCV Parameters

(3 categories, numeric parameters)

A
  1. Microcytic < 80 fl
  2. Normocytic 80-100 fl
  3. Macrocytic > 100 fl
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50
Q

Fill in the Chart

A
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51
Q

Macrocytic Anemias

(list 2, alternate name - kind of-, and general MOA)

A

Name: Megaloblastic Anemias

Included Conditions:

  1. Vitamin B12 deficiency
  2. Folic acid deficiency

MOA: Both B12 and folic acid contribute to DNA synthesis. Without these “ingredients” it takes much longer for erythroblasts to reach nuclear maturity. Meanwhile, RNA responsible for cytoplasmic proliferation is functional. While nuclear progression is retarded, cytoplasmic is not. This creates cells with excess cytoplasm, disturbing erythrocytic strength and oxygen carrying capacity.

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52
Q

Define Vitamin B12 (Cobalamine) Deficiency Anemia

A

Anemia due to inadequate supply of vitamin B12 for erythrocyte DNA synthesis

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53
Q

Etiology, Vitamin B12 (Cobalamine) Deficiency Anemia

(4 causes)

A
  1. Pernicious anemia (autoimmunity directed at parietal cells)
  2. Strict vegan diet (decreased B12 intake)
  3. Gastric surgery (bypass parietal cells)
  4. Crohn’s Disease
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54
Q

Pathophysiology, Vitamin B12 (Cobalamine) Deficiency

(2 steps, 3 causes of step 1)

A
  1. Inadequate B12 absorption due to
    • Lack/dysfunction/bypass of terminal ilium parietal cells responsible for B12 absorption
    • Lack of intrinsic factor (IF) in parietal cells
    • Lack of B12 intake
  2. Leads to impaired erythroblast DNA assembly, despite normal cytoplasm proliferation. Results in fragile macrocytic cells c poor oxygen carrying capacity.
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55
Q

S/Sx, Vitamin B12 (Cobalamine) Deficiency Anemia

(1 symptom, 4 signs)

A

Symptoms

  • Typical anemia symptoms

Signs

  • Glossitis c burning sensation
  • Neuro dysfunction
    • Stocking glove parathesia
    • Loss of sensation in position, fine touch, vibration
    • Clumsiness and ataxia
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56
Q

Differential Dx, Vitamin B12 (Cobalamine) Deficiency

A

Folic Acid Deficiency Anemia

Difference - B12 presence of neurological symptoms

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57
Q

Diagnostic Tests, Vitamin B12 (Cobalamine) Deficiency

(4 tests)

A
  1. CBC
    • Severe anemia
    • MCV >100 (macrocytic)
    • Hypersegmented neutrophils
    • Anisocytosis
    • Poikilocytosis
    • Howell Jolly bodies
  2. Vitamin B12 assay = decreased
  3. Folate assay = normal
  4. Shilling test – antiquated
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58
Q

Medical Tx, Vitamin B12 Deficiency Anemia

(4)

A
  • Vitamin B12 supplimentation
    • First month = weekly IM injections
    • Remainder of life = monthly IM injections, 1000 mcg
    • *Overwhelming the system c B12 will work even in pts lacking IF. This is due to passive diffusion against the B12 concentration gradient in the event of excess B12 *
  • Oral cobalamin, 0.5-2 mg/day
    • Only if pt is lacking B12 intake due to necessity of functional parietal cells to absorb
  • OTC SQ tablet, not as effective
  • Nightly nasal spray, not as effective and expensive
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59
Q

Pt Education/Prevention, Vitamin B12 (Cobalamine) Deficiency Anemia

(2)

A
  1. Hemostalogic correction usually occurs within 6 weeks
  2. Neurologic improvement may take up to 18 months, if at all
    • Reactivity to tx depends on how long neuro symptoms have been present. Usually, < 6 mo has positive prognosis
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60
Q

Provider Pearls, Vitamin B12 (Cobalamine) Deficiency Anemia

(2)

A
  1. Patients may try to self-treat the glossitis c oral rinses. Since this is a neuro sign the rinces will not work but the bacteria in the mouth will be eliminated, often resulting in Candida
  2. Cells may never appear macrocytic. In suspicion of B12 anemia, the most reliable test is the B12 assay
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61
Q

Define Folic Acid Deficiency Anemia

A

Inadequate supply of vitamin B12 for erythrocyte DNA synthesis

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62
Q

Etiology, Folic Acid Deficiency Anemia

(4 causes)

A
  1. Poor dietary intake
    • Alcoholics
    • Anorexia (psychological or secondary to comorbidity like cancer)
    • Diet low in citrus fruits and dark leafy greens
  2. Malabsorption (celiac, Crohn’s)
  3. Pregnancy
  4. Hemolytic anemia
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63
Q

Pathophysiology, Folic Acid Deficiency Anemia

A

Inadequate folic acid absorption leads to impaired erythroblast DNA assembly, despite normal cytoplasm proliferation. Results in fragile macrocytic cells c poor oxygen carrying capacity.

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64
Q

S/Sx, Folic Acid Deficiency Anemia

(1 symptom, 3 main signs - 1 has 4 aspects)

A

​Signs

  1. Glossitis
  2. Vague GI complaints
    • Anorexia
    • Nausea
    • Abd Pn
    • Constipation/diarrhea
  3. No neurologic s/sx

Symptoms Typical to general anemia

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65
Q

Differential Dx, Folic Acid Deficiency Anemia

(1)

A

B12 (Cobalamine) deficiency anemia

*It is likely folic acid if there are no neuro s/sx and the pt is an alcoholic *

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66
Q

Diagnositc Tests, Folic Acid Deficiency Anemia

(3)

A
  • CBC
    • Severe anemia
    • MCV >100 (macrocytic)
    • Macro-ovalocytes
    • Hypersegmented neutrophils
    • Howell Jolly bodies
  • Vitamin B12 assay = normal
  • Folate assay = decreased
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67
Q

Medical Tx, Folic Acid Deficiency Anemia

(3)

A
  1. Folic acid 1 mg po daily
  2. Avoid alcohol
  3. Avoid folic acid antagonists, or prophylactically supplement if avoidance is unreasonable
    • Methotrexate
    • Trimethoprim sulfate (antibiotic)
    • Phenytoin (dilantin)
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68
Q

Pt Education/Prevention, Folic Acid Deficiency Anemia

A

Pregnant women c folic acid anemia may lead to fetuses c neural tube defects. Recommendation: start folic acid tx 3 mo before conceiving

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69
Q

Vitamin B12 (Cobalamine) Sources

A

Animal products

  1. Red meat
  2. Poultry
  3. Dairy

The body also produces some B12 and stores amounts to last for months (2-6) before s/sx of B12 deficiency anemia present

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70
Q

Define Pernicious Anemia

A

Anemia resulting from lack of intrinsic factor production in the stomach. This decreases Vitamin B12 (cobalamine) absorption in the ilium, eventually causing Vitamin B12 Deficiency Anemia

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71
Q

Stocking Glove Deformity

(define, 2 associated conditions)

A

Neurologic condition demonstrated by parasthesia on the hands and feet in the shape of a glove or stocking.

Associated Conditions

  1. Vitamin B12 (Cobalamine) Deficiency
  2. Diabetic Neuropathy
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72
Q

Hypersegmented Neutrophil

A

Neutrophil c > 8 nuclear lobuoles, present in megaloblastic anemias.

Exact MOA for this finding is up for debate but it is the landmark for this group of anemias.

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73
Q

Howell Jolly Bodies

(define, significance in hemotology)

A

Definition: RBC inclusion bodies representing DNA fragments of dysfunctional nucleus. Cells with Howell Jolly Bodies in the presence of megaloblastic anemia may also be macrocytic

Signif: Identifying factor for megaloblastic anemias

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74
Q

Dietary Folic Acid Sources

(2)

A
  1. Dark, leafy greens
  2. Citrus fruits
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75
Q

Hemolytic Anemia Classifications

(2, 3 disorders per category)

A
  1. Intrinsic
    • Cell membrane disorders (spirocytosis)
    • Enzyme system disorders (for cell membrane formation)
    • Hgb disorders
  2. Extrinsic
    • Autoimmune disorders (commonly RA, lupus)
    • Microangiopathic hemolytic disorders (shows RBC fragments)
    • Malaria
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76
Q

What do greasy stools indicate?

A

Folic Acid Deficiency Anemia, when in conjunction c other anemia s/sx

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77
Q

Common Dx Tests and Results for Hemolytic Anemias

(4)

A

Note - these results are often encountered after obtaining a fairly normal CBC

  1. Decreased haptoglobin, usually
    • Hemoglobin carrier. If you have RBC destruction then more of these are full so the levels are low
  2. Increased indirect bilirubin
    • Bliiverdin is precursor for blilrubin. This is free bilirubin in the blood, unbound
  3. Increased total bilirubin
    • Includes indirect bilirubin and direct bilirubin – bound bilirubin
  4. Increased LDH
    • Lactate dehydradinase – released from broken RBC
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78
Q

Intrinsic Hemolytic Anemias

(define, list 3)

A

Definition: RBC destruction is due to decrease in the RBC itself

Intrinsic Hemolytic Anemias:

  1. Hereditary Spherocytosis
  2. G6PD Deficiency
  3. Sickle Cell Disease
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79
Q

Define Hereditary Spherocytosis

A

Genetic hemolytic anemia due to fragile spherical erythrocytes

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80
Q

Etiology, Hereditary Spherocytosis

A

Autosomal dominant disorder

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81
Q

Pathophysiology, Hereditary Spherocytosis

(3 steps)

A
  1. RBCs produce deficient number of membrane PRO
  2. RBC form c decreased surface:volume ratio and fragile spherical shape (spherocytes)
  3. Decreased flexibility causes hemolysis when passing through spleen
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82
Q

S/Sx, Hereditary Spherocytosis

(2 symptoms, 3 signs)

A

Symptoms

  • RUQ Pain
  • Family history

Signs

  • Jaundice noted by scleral icterus
  • Gallstones c predominating bilirubin crystal composition
  • Palpable spleen
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83
Q

Differential Dx, Hereditary Spherocytosis

(5)

A

Other hemolytic anemias

  1. G6PD deficiency
  2. Sickle cell disease
  3. Autoimmune hemolytic anemia
  4. Microangiopathic hemolytic anemia
  5. Aplastic anemia
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84
Q

Diagnostic Tests, Hereditary Spherocytosis

(4 tests)

A
  • CBC
    • Variable anemia c aplastic crises concurrent c BM stress
    • Dimorphic population including spherocytes
    • Microcytosis c increased MCHC (>36 g/dl)
    • Reticulocyte count increased
  • Indirect bilirubin increased
  • **Osmotic fragility test – positive **
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85
Q

Medical Tx, Hereditary Spherocytosis

A

Folic acid x 1 mg/d

*Explaination - *

  • Folic acid helps inc RBC count by quickening nuclear maturation
  • Usually these pts live c a dimorphic cell population. Folic acid helps to push dimorphism to favor normal RBCs
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86
Q

Surgical Tx, Hereditary Spherocytosis

A

Splenomegaly

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87
Q

Spherocyte

(definition, conditions creating spherocytes)

A

Fragile, extra round RBC that is formed due to a decreased SA; lacking cell membrane to make bionclave disk. These cells are very fragile and often lyse with very little physical stress (ex - entering the spleen). Due to the decrease in SA, spherocytes posess deficient oxygen carrying capacity

Present in:

  1. Hereditary spherocytosis
  2. Following a blood transfusion
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88
Q

Bilirubin and Hemolytic Anemias

(2 signs, their MOAs)

A
  1. Scleral icterus (jaundice seen in the eyes)
    • Excess hemolysis
    • Increased bilirubin secretion to aid in RBC byproduct excretion
    • Excessive bilirubin circulation in bloodstream
    • Jaundice
    • Scleral icterus
  2. Cholelithiasis
    • Excess hemolysis
    • Increased bilirubin secretion to aid in RBC byproduct excretion
    • Excessive bilirubin traveling from bile duct
    • Occlusion of bile ducts
    • Gallstones
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89
Q

Osmotic Fragility Test

(Indication, Procedure)

A

Indication: suspected presence of spherocytes

Procedure: Place RBC in hypotonic soln
•Normally, cell membranes can withstand this and swell
•Spherocytes cannot stand the swelling and they burst

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90
Q

Splenectomy in Hemolytic Anemia

(explain this tx)

A

The spleen is usually the site of hemolysis of fragile cells associated c hemolytic anemia. Splenic elimination allows for these cells to persist and carry O2, even if their carrying capacity is less than healthy RBCs

Liver can compensate and filter blood in the absence of the spleen

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91
Q

Define G6PD Deficiency

A

Episodic hemolytic anemia due to glucose 6 phosphate dehydrogenase deficiency

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92
Q

Etiology, G6PD Deficiency

(1 cause, 3 stressors - 1 c 4 specifics)

A

Y-linked disorder, common among 10-15% of African black men who encounter an oxidative stress:

  • Infection (viral or bacterial)
  • Hypoxia (often from pneumonia)
  • Drugs
    • Quinidine
    • Sulfa drugs (trimethoprim sulfa)
    • Nitofurantoin (macrodantin)
    • Asprin
    • Many other drugs, but you will usually have the ability to look these up in clinic. These are just the most commonly used ones

Board Question - sulfa drugs and G6PD crisis

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93
Q

Pathophysiology, G6PD Deficiency

(5 steps)

A
  1. RBC lose G6PD as they age
  2. Lack of G6PD decreases RBC’s ability to deal c oxidative stress (crisis)
  3. Oxidized hgb denatures to form Heinz bodies
  4. Heinz bodies formation damages the membrane
  5. Damaged RBCs removed by spleen
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94
Q

S/Sx, G6PD Deficiency

(2 symptoms, 3 signs)

A

Symptoms

Asymptomatic until oxidative stress. Stress induces

  1. SOB
  2. Organ specific hypoxia complaints

Signs

Without signs until oxidative stress. Stress induces

  1. Hypoxia
    • SOB
    • Cyanosis
  2. Jaundice noted by scleral icterus
  3. Organ specific s/sx related to hypoxia
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95
Q

Differential Dx, G6PD Deficiency

(5)

A

Other hemolytic anemias

  1. Hereditary spherocytosis
  2. Sickle cell disease
  3. Autoimmune hemolytic anemia
  4. Microangiopathic hemolytic anemia
  5. Aplastic anemia
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96
Q

Diagnostic Tests, G6PD Deficiency

(4)

A
  • CBC and blood smear
    • **Heinz bodies **abnl RBC shape from hemoglobin denaturation in crisis/age
    • **Bite cells **Heinz bodies with the inefficient proteins removed (by the spleen)
  • Reticulocyte count increased
  • Indirect bilirubin increased
  • G6PD assay
    • **During crisis – non revealing **(crisis stimulates RBC production. When first formed, cells have greater [G6PD])
    • Weeks after crisis – deficient (this is when the cells age and rapidly lose G6PD)
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97
Q

Medical Tx, G6PD Deficiency

A

Remove source of hypoxia

*This could be emergent depending on crisis serverity *

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98
Q

Define Sickle Cell Disease

A

Chronic illness resulting in anemia from abnormal hemoglobin

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99
Q

Etiology, Sickle Cell Disease

A

Autosomal recessive genetic disorder where valine substitutes for glutamine on beta chain. Most common in Africans (1/400 are homozygous)

Hemoglobinopathy Variations:

  • Hgb SS – sickle cell disease
  • Hgb AS – sickle cell trait
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100
Q

Pathophysiology, Sickle Cell Disease

(4 steps)

A
  1. Infants are born normally c predominating Hgb F levels
  2. S/sx appear as Hgb f is replaced by abnormal Hgb S
  3. Underlying anemia due to splenic attempt to clear abnormal cells
  4. Crises – Upon oxidative stress Hgb S crystallizes and sickles RBC shape. These get caught throughout system causing pain and potential organ swelling, dysfunction, and infarction
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101
Q

Symptoms, Sickle Cell Disease

(3, 3 common sites)

A

Asymptomatic unless in crisis. Crisis symptoms may occur in varying frequency/duration and are characterized as acute pain due to vaso-occlusive episodes:

  • Hypoxia
  • Infarction
  • Dehydration (manifested in stomach pn)

Common pain sites

  • Spine
  • Long bones (esp distal portions c smaller BV)
  • Chest
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102
Q

Signs, Sickle Cell Disease

(include 6 signs c 8 consequence)

A

Asymptomatic unless in crisis. Crisis signs may occur in varying frequency/duration and are characterized as

  • Jaundice
  • Pigment gallstones
  • Splenohepatomegaly (esp in peds)
  • Blood pooling in extremities – edema/ecchymosis/ulcer
  • +/- low grade fever
  • Vaso-occlusive crisis may lead to
    • CVA
    • Priapism
    • MI
    • PE
    • Ischemic necrosis
      • Liver
      • Bone – Staph related osteomyelitis

Permanent anatomic changes due to repeated crises

  • Poorly healing ulcers overlying distal tibia
  • Scull “towering” and “bosses”

Associated Mortality

  • Infections
  • Pulmonary emboli
  • Occlusion of vital vessel
  • Renal failure

*Crisis stimulation for sickle cell trait is much more extreme – ie rigorous exercise at high altitude *

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103
Q

Differential Dx, Sicle Cell Disease

(5)

A

Other hemolytic anemias

  1. Hereditary spherocytosis
  2. G6PD deficiency
  3. Autoimmune hemolytic anemia
  4. Microangiopathic hemolytic anemia
  5. Aplastic anemia
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104
Q

Diagnostic Tests, Sickle Cell Disease

(5 tests)

A
  1. CBC
    • Hct increased
    • WBC count increased – 12,000-15,000/mcl
      • ​Due to inc physiologic or pathologic stress
  2. Peripheral blood smear
    • 5-50% RBC are sickled
    • **Nucleated RBC’s **(polychromasia)
    • Howell Jolly bodies (export immature RBC to compensate for hemolysis)
    • Target cells (underlying hypochromic anemia)
  3. Reticulocyte count increased
  4. Bilirubin assays – both total and indirect increased
  5. Hbg electrophoresis –confirmatory test
    • Sickle Cell Disease
      • Hgb S 85-98%
      • Hgb F 5-15%
      • Hgb A2 1-3%
      • Hgb A 0%
    • Sickle Cell Trait
      • Hgb A 60%
      • Hgb S 40%
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105
Q

Medical Tx, Sickle Cell Disease

(4)

A
  • Folic acid supplementation
  • Pneumococcal vaccine (prevent strep pneumo, a common cause for crisis)
  • Transfusions as needed
  • Hydroxyurea to inc. Hgb F levels
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106
Q

Surgical Tx, Sickle Cell Disease

A

Allogenic hematopoietic stem cell transplant from sibling donor

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107
Q

Emergency Tx, Sicke Cell Diseaes

(5)

A

Crisis Tx

  1. Vigorous oral or IV hydration
  2. Analgesics (morphine)
  3. Narcotics
  4. Treat precipitating factor – Oxygenate for hypoxia
  5. Blood transfusions
    • If Hgb < 5mg/dl
    • Cardiac failure
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108
Q

Pt Education/Prevention, Sickle Cell Disease

(2)

A

Life expectancy for disease = 40-50 years

Behavior change to avoid crises if possible

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109
Q

Prevelence, Sickle Cell Disease

A

1/400 African black people

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110
Q

Age of Presentaion - Sickle Cell Disease

(5 ages, % of diagnosed pts)

A
  • Six months of age — 6 percent
  • Twelve months of age — 32 percent
  • Two years of age — 61 percent
  • Six years of age — 92 percent
  • Eight years of age — 96 percent
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111
Q

Extrinsic Hemolytic Anemias

(Define, List 4)

A

Definition: Hemolytic anemia characterized by destruction of normal RBC by another source (it isn’t the RBC’s problem, it is the body’s problem)

Extrinsic Anemias:

  1. Autoimmune Hemolytic Anemia
  2. Microangiopathic Hemolytic Anemia
  3. Malaria
  4. Drugs
    • Nitrofurantoin (uti antibiotic)
    • Sulfonamide (uti antibiotic)
    • Chloramphenicol (anticancer)
    • Primaquine (anti-malarial)
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112
Q

Define Autoimmune Hemolytic Anemia

A

Aquired anemia caused by IgG autoantibody attack on normal RBC

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113
Q

Etiology, Autoimmune Hemolytic Anemia

A
  • 50% are idiopathic
  • Other cases associated c
    • SLE (lupus)
    • CLL
    • Lymphomas
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114
Q

Pathophysiology, Autoimmune Hemolytic Anemia

(5 steps)

A
  1. IgG binds to RBC
  2. Antigen/antibody complex interacts c splenic macrophages
  3. Macrophages remove only that portion of the RBC membraine
  4. RBC leaves spleen as spherocyte
  5. Spherocyte fragile either spontaneously lyse or are removed by spleen
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115
Q

S/Sx, Autoimmune Hemolytic Anemia

(2 symptoms, 4 signs)

A

Symptoms

  • Acute onset
  • Fatigue

Signs

  • Jaundice
  • Pallor
  • Gallstones
  • Splenomegaly
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116
Q

Diagnostic Tests, Autoimmune Hemolytic Anemia

(4)

A
  1. CBC
    • Variable anemia
    • Spherocytes
  2. Reticulocyte count increased
  3. Indirect bilirubin increased
  4. Coombs test (direct antiglobulin) positive
    • Indirect Coombs is + or -
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117
Q

Medical Tx, Autoimmune Hemolytic Anemia

(3, in order of progression)

A

Do not move on until tx fails

  1. Prednisone (divided doses totaling 1-2 mg/kg/day)
    • ​​Retards immune response
  2. Blood transfusion if Hct <30%
  3. Immunosuppressive agents
    • Rituxamib - antibody againt B cell antigen
    • Cyclosporine - *suppress cell mediated immune response *

Common PANCE question - Prednisone is drug of choice

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118
Q

Surgical Tx, Autoimmune Hemolytic Anemia

(2)

A
  1. Splenectomy if pt is refractory to prednisone
  2. Plasmapheresis in pts c rapid hemolysis
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119
Q

Direct and Indirect Coombs Tests

(definitions, use)

A

Defintions:

  • Coombs Test - mix RBC c Coombs reagent (IgG) to test for agglutination
  • Indirect Coombs - evaluate plasma or serum for abnormal antibodies

Uses:

  • Coombs Test - diagnose autoimmune hemolytic anemia
  • Indirect Coombs - not for diagnosing autoimmune hemolytic anemia
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120
Q

Plasmapheresis

(def, function)

A

Definition: Replace all of the plasma in a person’s body. Requires a special machine, reserved for highly severe cases

Use: Tx autoimmune hemolytic anemia for pts c rapid hemolysis

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121
Q

Define Microangiopathic Hemolytic Anemia

A

Group of disorders characterized by RBC fragmentation

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122
Q

Etiology, Microangiopathic Hemolytic Anemia

(6)

A
  1. Disseminated intravascular coagulopathy
  2. Thrombotic thrombocytic purpura
  3. Hemolytic uremic syndrome
  4. Malfunctioning cardiac valve prosthesis
  5. Malaria
  6. Drugs
    • Nitrofurantoin
    • Sulfonamide
    • Chloramphenicol
    • Primaquine
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123
Q

Pathophysiology, Microangiopathic Hemolytic Anemia

A

Variable source of fragmentation; most popular is RBC interaction c abnormal intravascular fibrin strands. RBC fragments are not useful and are disposed of in spleen. Paired c coagulopathy and thrombocytopenia.

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124
Q

S/Sx, Microangipathic Hemolytic Anemia

(1 sign, 1 symptom)

A

Both signs and symptoms depend on underlying cause. Heart-valve related cases may resemble iron deficiency

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125
Q

Differential Dx, Microangioplastic Hemolytic Anemia

(6)

A
  • Iron deficiency anemia
  • Other hemolytic anemias
    • Hereditary spherocytosis
    • G6PD deficiency
    • Sickle cell disease
    • Autoimmune hemolytic anemia
    • Aplastic anemia
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126
Q

Diagnostic Tests, Microangioplastic Hemolytic Anemia

(2)

A
  1. Peripheral Smear indicates fragmented RBCs
  2. CBC may reveal decreased Hct and Hgb
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127
Q

Medical Tx, Microangioplastic Hemolytic Anemia

(1)

A

Treat the underlying disorder

128
Q

Define Aplastic Anemia

A

Pancytopenia due to bone marrow failure

129
Q

Etiology, Aplastic Anemia

(7)

A
  1. Idiopathic
  2. SLE (lupus) or any autoimmune disease
  3. Chemotherapy
  4. Radiation therapy
  5. Toxins
    • Benzenes (organic)
    • Toluenes (organic)
    • Insecticides (inorganic)
  6. Drugs
    • Chloramphenicol
    • Sulfonamides
    • Phenytoin
    • Carbamazepine
  7. Pregnancy (uncommon)
130
Q

Pathophysiology, Aplastic Anemia

A

Hematopoeitic stem cell injury/abnormal expression causes defects in all blood cell lines

131
Q

S/Sx, Aplastic Anemia

(2 symptoms, 5 signs in 3 categories)

A

Symptoms Anemia effects

  • Fatigue
  • Weakness

Signs

  • Anemia effects
    • Pallor
  • Leukopenia effects
    • Increased infections
  • Thrombocytopenia effects
    • Mucosal/skin bleeding
    • Purpura
    • Petechiae
132
Q

Differential Dx, Aplastic Anemia

(3, 1 has 3 specifics)

A
  1. Increased Blasts - Acute Leukemia
  2. Sepsis
  3. Normomarrow pancytopenia
    • SLE (lupus)
    • Hypersplenism
    • Disseminated infection
133
Q

Diagnostic Tests, Aplastic Anemia

(3)

A
  1. CBC
    • Pancytopenia
    • Blast cells decreased
  2. Reticulocyte count decreased
  3. Bone marrow biopsy hypocellular
134
Q

Medical Tx, Aplastic Anemia

(4)

A

Refer to hematologist, who will do the following

  1. Blood Transfusion
  2. Platelet transfusion
  3. Antibiotics
  4. Cyclosporine and antithymocyte globulin (from rabbits)
    • Tx of choice for pts > 50 yo
135
Q

Surgical Tx, Aplastic Anemia

A

Allogenic bone marrow transplant if < 50 yo

136
Q

“Aplastic”

A

“Without plasticity,” meaning inability to mold/shape/produce new cells

137
Q

Patient Ed/Prevention, Aplastic Anemia

A

Good long term prognosis – partial response in 70% of cases

138
Q

Leukocyte Classifications

A
  1. Granulocytes
    • Neutrophils
    • Basophils
    • Eosinophils
  2. Monocytes
  3. Lymphocytes
139
Q

Granulocyte Maturation

(

A

Complete development in BM

  1. Blast cell
  2. Myelocyte
  3. Band Cell (some of these do enter circulation; 3-5% of all circulating WBC are band cells)
  4. Mature WBC
140
Q

Macrophage Maturation

A
  1. Begin differentiation from hematocytoblast in BM
  2. Enter peripheral blood upon becoming a monocyte
  3. Circulate for 12-24 hours, until it takes residency in tissue
  4. Differentiate into macrophage
141
Q

Lyphocyte Maturation

A
  1. Lymphoid stem cells that are stimulated by antigens precurse all lymphocytes
  2. Cells separate depending on how they differentiate
    • B-cell and NK cells remain in the BM to mature
    • T-cells migrate to peripheral lymphoid tissues
      • Thymus
      • Spleen
      • Tonsils
      • Lymph nodes
142
Q

Neutrophils

(3 alternate names, function, indication for concentration change)

A

Names: segs, polys, PMN’s

Function: First line of defense, phagocytize pathogens and foreign particles

Changes: Usually 55-65% WBCs

  • Increased - bacterial infections. Often increased c higher [band cell] for left shift (paired c metamyelocytes and myelocytes))
  • Decreased - chemotherapy or aplastic anemia
143
Q

Lymphocytes

(functions, changes in number)

A

Functions:

  1. Cell mediated immunity - T lymphocytes (CD4, CD8, supressors), NK cells
  2. Humoral immunity - B lymphocytes → plasma cells → antibodies

Changes: Usually 20%, second most popular lymphcyte

  • Increased - lymphocytic leukemia, viral infections
  • Decreased - HIV
144
Q

Reactive/atypical lymphs

A

Cells that assist c infectious mono/Epstein Barr virus

Note - this will not be paired c monocyte elevation

This is often a board question and Sandy will most likely ask this on the test

145
Q

Monocytes

(function, normal concentration, 4 macrophage types)

A

Function: Circulating phagocytes, present antigens to T lymph for immune response

Normal concentration: 5-10% WBC

Macrophage types:

  1. Kupffer cells - liver
  2. Alveolar macrophages - lungs
  3. Histiocytes - CT
  4. Osteoblasts - bone
146
Q

Leukemia

(gen. def, classification system)

A

Definition: Characterized by unrestricted growth of leukocytes and leukocyte precursors

  • Cells are nonfunctional in providing usual protection against infection

Classification based on:

  • Cell type
    • Myeloid
    • Lymphoid
  • Presentation
    • Acute
    • Chronic
147
Q

Leukemia Risk Factors (4)

A
  1. Family hx
  2. Exposure to ionizing radiation, forming dangerous free radicals
  3. Benzene
  4. Some alkylating agents
  5. Cyclophosphamide
    1. Anticancer drug that undergoes hydroxylation à active compound reacts with DNA leading to cytotoxicity
148
Q

Pathophysiology, Leukemia (general)

A

Replacement of normal bone marrow with malignant cells

This is the basis for all s/sx. Logic!

149
Q

Acute Leukemias

(list 2)

A
  1. ALL - Acute Lymphocytic Leukemia
  2. AML - Acute Myelogenous Leukemia
150
Q

Define Acute Lymphocytic Leukemia

A

Acute unrestricted growth of lymphocytes and their precursors

151
Q

Etiology, Acute Lymphocytic Leukemia

A
  • Most common pediatric leukemia (80%), years 3-7
  • Adult, 20% of leukemias, usually posess a risk factor
    • Family hx
    • Ionizing radiation exposure
    • Benzene
    • Some alkylating agents (cyclophosphamide)
  • Prognosis varies upon
    • Age
    • WBC
    • Presence of Philadelphia chromosome
152
Q

Pathophysiology, Acute Lymphocytic Leukemia

A

Mutation to only the lymphocyte precursor. Produces excess and ineffective T, B, and NK cells. Excess cells “clog” the body and do not

153
Q

S/Sx, Acute Lymphocytic Leukemia

(5 symptoms, 4 signs)

A
  • Occur over days to weeks*
  • Remember, all s/sx either pertain to ineffective BM or ineffective lymphocytes*

Symptoms Reflective of dysfunctional lymphoid cells

  • Fatigue
  • Lethargy
  • HA
  • Bone pain
  • Joint pain

Signs Reflective of dysfunctional lymphoid cells

  • Fever
  • Pallor
  • Lymphadopathy
  • Hematosplenomegaly
154
Q

Differential Dx, Acute Lymphocytic Leukemia

(4)

A
  1. CLL
  2. Aplastic Anemia
  3. Bacterial Infection
  4. Sepsis
155
Q

Diagnostic Tests, Acute Lymphocyte Leukemia

(4)

A
  1. CBC – high WBC count (lymphocytosis)
  2. Bone marrow biopsy > 20% nucleated BM cells are lymphoblasts
    • Confirmatory test
  3. CXR – mediastinal mass
  4. Genetic testing – Philadelphia chromosome sometimes present
    • Unfavorable dx

Notes

  • Lymphoblast can be definitively named in BM but not in peripheral circulation
  • Mediastinal mass is from clogged lymph nodes
  • Philidelphia chromosome is not a landmark for this leukemia but it will change your course of tx due to a change in likely disease progression
156
Q

Medical Tx, Acute Lymphocytic Leukemia

A

Itensive Chemotherapy

  • Cycles of many txs and recovery
  • Induces pancytopenia
157
Q

Pt Education/Prevention, Acute Lymphocytic Leukemia

(1 point)

A

Prognosis – remission rate >70%

158
Q

Define Acute Myelogenous Leukemia

A

Acute unrestricted growth of myelocytes and their precursors, characterized by short duration of s/sx

159
Q

Etiology, Acute Myelogenous Leukemia

A
  1. Age – primarily a leukemia of people >60 yo
  2. Risk factors
    • Family hx
    • Ionizing radiation exposure
    • Benzene
    • Some alkylating agents (cyclophosphamide)
160
Q

Pathophysiology, Acute Myelogenous Leukemia

A

Mutation to only the myelocyte precursor. Produces excess and ineffective granulocytes, monocytes, thrombocytes, and erythrocytes. Excess cells “clog” the body and disturb all cell lines

161
Q

S/Sx, Acute Myelogenous Leukemia

(4 symptoms, 5 signs)

A
  • Both reflective of dysfunctional myeloid cells and bone marrow (note lack of infectious problems due to functioning lymphocytes)*
  • Onset time, days to weeks*

**Symptoms **

  • Bleeding
    • GI
    • Epistaxis
    • Skin
    • Menorrhagia
  • Bacterial infections
  • Joint pain
  • Bone Pain

Signs

  • Pallor
  • Gingival hyperplasia
  • Purpura
  • Petechiae
  • Lymphadopathy
162
Q

Differential Dx, Acute Myelogenous Leukemia

(4)

A
  1. CML
  2. Aplastic Anemia
  3. Bacterial Infection
  4. Sepsis
163
Q

Diagnostic Tests, Acute Myelogenous Leukemia

(3)

A
  1. CBC – high WBC count (leukocytosis)
  2. Bone marrow biopsy > 20% nucleated BM cells are myeloblasts
    • Confirmatory test
    • Auer Rods
  3. Peripheral smear contains myeloblasts
164
Q

Medical Tx, Acute Myelogenous Leukemia

A

Chemotherapy

165
Q

Surgical Tx, Acute Myelogenous Leukemia

A

BM Transplant

166
Q

Pt Ed/Prevention, Acute Myelogenous Leukemia

A

Prognosis – c chemotherapy, complete remission in (depending on severity)

  • 80-90% pts < 60 yo
  • 50-60% pts > 60 yo
167
Q

Chronic Leukemias

(list 2)

A
  1. Chronic Lymphocytic Leukemia
  2. Chronic Myelogenous (Myeloid) Leukemia
168
Q

Define Chronic Myelogenous Leukemia

A

Myeloproliferative disorder of extended duration

169
Q

Etiology, Chronic Myelogenous Leukemia

A
  • Young to middle aged adults (median age 55)
  • Risk factors
    • Family hx
    • Ionizing radiation exposure
    • Benzene
    • Some alkylating agents (cyclophosphamide)
170
Q

Pathophysiology, Chronic Myelogenous Leukemia

A

Abnormal myeloid cell production c normal cell differentiation. Bone marrow function persists in early disease. Occurs in three phases

  1. Chronic
    • Normal BM function
  2. Accelerated
    • Impaired neutrophil differentiation
    • WBC uncontrollable by chemo
  3. Acute (blast crisis)
    • Myeloid blast proliferation
    • Resembles AML
171
Q

S/Sx, Chronic Myelogenous Leukemia

(5 symptoms, 2 signs)

A

Symptoms

  • Fatigue
  • Weight loss
  • Low grade fever
  • Night sweats
  • Abdo fullness

Signs

  • Splenomegaly
  • Sternal tenderness
172
Q

Differential Dx, Chronic Myelogenous Leukemia

(5)

A
  1. AML
  2. CLL
  3. Bacterial Infection
  4. Sepsis (lesser WBC count than CML)
  5. Anemia, in later progression (not enough room in BM to produce funtional RBCs)
173
Q

Dx Tests, Chronic Myelogenous Leukemia

(2)

A
  1. CBC
    • Increased WBC (leukocytosis median count 150,000/mcl) c severe left shift
      • ​Considered CML at 50,000
    • Anemia – present in later stages
    • Thrombocytosis – present in later stages
  2. Genetic testing
    • Philadelphia chromosome (95% of the time)
    • Bcr/abl gene*
174
Q

Medical Tx, Chronic Myelogenous Leukemia

A

Refer to hematologist, who will do the following

  1. First line – Imatinib
    • Inhibits tyrosine kinase of bcr/abl oncogene
    • Controls chronic disease
  2. Second line – Dasatinib
    • More potent
    • Effective against bcr/abl mutations
175
Q

Surgical Tx, Chronic Myelogenous Leukemia

A

Third line – BM transplant

176
Q

Pt Ed/Prevention, Chronic Myelogenous Leukemia

A

Imatinib often achieves remission x 8 years

177
Q

Clinical Pearls, Chronic Myelogenous Leukemia

A
  • Pts c unexplained leukocytosis < 50,000 can be referred to hematology/oncology for monitoring. They may start chemo at lower threshold
  • When patients present to in blast crisis prioritize as follows:
    1. Achieve remission or at least reduce WBC count
    2. Figure out exact cause
178
Q

Philidelphia Chromosome

(define, 1 landmark associated condition)

A

Define - translocation in chromosomes 9 and 22

Disease - Chronic Myeloid Leukemia (present in 95% of cases)

179
Q

CML vs. Infection

(compare 5 factors)

A
180
Q

Define Chronic Lymphocytic Leukemia

A

AKA B Cell Leukemia

Clonal malignancy of B lymphocytes

181
Q

Etiology, Chronic Lymphocytic Leukemia

A

Most prevalent leukemia

  • Median age > 70 yo
  • Risk factors
    • Family hx
    • Ionizing radiation exposure
    • Benzene
    • Some alkylating agents (cyclophosphamide)
182
Q

Pathophysiology, Chronic Lymphocytic Leukemia

A

B lymphocyte clone will uncontrollably proliferate, creating ineffective B cells, thus ineffective plasma cells and antibodies.

Note – B cell only, not entire lymphocyte line

183
Q

S/Sx, Chronic Lymphocytic Leukemia

(2 symptoms, 2 signs)

A

Often detected when asymptomatic

Symptoms

  • Fatigue
  • Recurrent infections

Signs

  • Splenomegaly (spleen is in overdrive, usually lymphocytes last for years and rate of destruction is low)
  • Lymphadopathy
184
Q

Differential Dx, Chronic Lymphocytic Leukemia

(4)

A
  1. ALL
  2. Infection
  3. CML
  4. Sepsis
185
Q

Diagnostic Tests, Chronic Lymphocytic Leukemia

(2)

A
  1. CBC
    • High WBC count (leukocytosis > 20,000/ml)
    • +/- anemia
    • +/- thrombocytosis
    • peripheral lymphocytosis
  2. Peripheral smear reveals smudge cells
186
Q

Medical Tx, Chronic Lymphocytic Leukemia

(3)

A
  1. Early disease – observation
  2. Tx indications – S/sx that interfere c quality of life
    • Progressive fatigue
    • Symptomatic lymphadopathy, anemia, thrombocytopenia
  3. Chemotherapy
    • Fludarabine - purine neucleotide analog
    • Rituxamib (Retuxan) - monoclonal antibody directed against CD20 antigen on normal and malignant B lymphocytes
187
Q

Surgical Tx, Chronic Lymphocytic Leukemia

A

Allogenic stem cell transplant for pts who do not respond to chemo

188
Q

Pt Ed/Prevention, Chronic Lymphocytic Leukemia

A

Mean survival time = 6 years. Choice to treat depends on quality of life and is completely up to the patient .

Prognoses:

  • Stages 0 or 1 = 10-15 years
  • Stages 3 or 4 = 2 years
189
Q

Smudge Cells

(def, associated condition)

A

Definition: Ruptured Lymphyocytes

Condition: this is a hallmark of CLL

Smude cell is pictured in square, normal lymphocytes in circles

190
Q

Most Prevelant WBC in Chronic Inflammation

A

Monocytes are first line

  • Other “big players” - neutrophils and lymphocytes*
  • (neutrophils will predominate in an acute flair up)*
  • Remember, they reside in the tissue. Basophils and eosinophils are not very numerous, so they are not a big player. *
191
Q

When may a Pt Possess High Eosinophil Count?

A

Parasites

192
Q

Define Hodgkin’s Lymphoma

A

Neoplasm (cancer) of the lymphatic system characterized by the presence of Reed Sternberg cells

193
Q

Etiology, Hodgkin’s Lymphoma

A
  • Largely unknown, although Epstein Barr virus is suspected
  • Bimodal age distribution (ages vary depending on source)
    • 15-45 yo c mean age in 20’s
    • > 50 yo
194
Q

Pathophysiology, Hodgkin’s Lymphoma

A
  • Tumor formation due to specific cell proliferation
    1. Normal lymphocytes (majority)
    2. Malignant B lymphocytes
    3. Reed Sternberg cells
  • Lymph node enlargement from cell infiltration
    1. Eosinophil
    2. Plasma cells
  • Presents in stages (don’t need these for our exam)
    1. Stage I – singular lymph node or extralymphatic site
    2. Stage II – 2+lymph nodes and/or extralymphatic tissue on ipsilateral side of diaphragm
    3. Stage III – lymph nodes on contralateral sides of diaphragm. May include spleen or extranodal disease
      • III1 – limited to upper abdomen (spleen, celiac, portohepatic nodes)
      • III2 – limited to lower abdomen (periaortic, pelvic, inguinal nodes)
    4. Stage IV – diffuse extralymphatic disease. Commom sites include
    • Liver
    • BM
    • Lung
    • Skin
195
Q

S/Sx, Hodgkin’s Lymphoma

(5 symptoms, 3 signs)

A

Symptoms

These are vague. Keep a high index of suspiscion when encountered c these symptoms

  1. Fever
  2. Night sweats
  3. Fatigue
  4. Weight loss
  5. Pruritis

Signs

  1. Painless, rock hard, swollen lymph nodes. Disease usually starts in the following nodes:
    • Cervical
    • Supraclavicular
    • Mediastinal
  2. Splenomegaly (contains lymphoid tissue)
  3. Potential hepatomegaly (contains lymphoid tissue)
196
Q

Differential Dx, Hodgkin’s Lymphoma

(4)

A
  1. Non-Hodgkin’s Lymphoma
  2. HIV
  3. Hypersensitivity Rxns
  4. Other solid tumors
197
Q

Diagnostic Tests, Hodgkin’s Lymphoma

(3 + 1 bonus)

A
  1. Lymph node biopsy
    • Reed Sternberg cells
    • Necrosis
    • Fibrosis
  2. Bone marrow biopsy
    • Lymphocytosis only present in progressive disease
  3. Scans to evaluate disease stage
    • CXR or chest CT
    • Abdo CT
    • Bone scan
  4. Bonus - US to evaluate cystic vs solid/complex
198
Q

Medical Tx, Hodgkin’s Lymphoma

(3 general, 2 specifics each)

A
  1. Chemotherapy and/or radiation (varies c histologic interpretation/staging)
    • AVBD – front line
    • MOPP – alternative
  2. Experimental – targeted therapies
    • Monoclonal antibodies
    • Target-specific aids
  3. Symptomatic tx
    • Antiemetics
    • Sedatives
199
Q

Surgical Tx, Hodgkin’s Lymphoma

A

Autologous bone marrow transplant

200
Q

Reed Sternberg Cell

(definition, associated condition)

A

Definition: gigantic, multi-nucleated, bilobed lymphocyte c characteristic “owl eye” formation

Associated Condition: Hodgkin’s Lymphoma

201
Q

Define Non-Hodgkin’s Lymphomas

A

Hematogenous group of malignancies originating in l ymph nodes and other lymph node tissues

202
Q

Etiology, Non-Hodgkin’s Lymphomas

A
  • Common pt populations
    • Autoimmune disorders
    • HIV
    • Immunosuppressive tx
  • Definitive etiology unknown. Some patterns in pts c
    • Viral etiology (virus may alter B cell)
    • Toxin exposure
      • Benzene
      • Pesticides
      • Herbicides
203
Q

Pathophysiology, Non-Hodgkin’s Lymphoma

(3 steps)

A
  1. Oncogene becomes juxtaposed next to
    • Immunoglobulin gene (B-cell lymphoma)
      • 85% of cases
    • T-cell receptor gene (T-cell lymphoma)
  2. Oncogene over-expression
  3. Excess T- or B-cell production, causing lymphoma
204
Q

S/Sx, Non-Hodgkin’s Lymphomas

(6 symptoms, 3 signs)

A

Symptoms

  • “B Symptoms” (present in B- and T-cell lymphomas)
    • Weight loss
    • Fever
    • Drenching night sweats
  • Other
    • Fatigue
    • Pruritis
    • Abdo pn

Signs

  • Uneven enlargement of tonsils and adenoids
  • Painless uneven lymphadenopathy
    • Cervical
    • Suprascapular
  • Dramatic infections
205
Q

Differential Dx, Non-Hodgkin’s Lymphomas

(2)

A
  1. Hodgkin’s Lymphoma
  2. Infectious mononucleosis
206
Q

Diagnostic Tests, Non-Hodgkin’s Lymphomas

(3 broad)

A
  1. Lymph node biopsy – No Reed Sternberg Cells
  2. Tissue biopsy or enlarged lymphatic tissues
    • Tonsils
    • Adenoids
  3. Scans to evaluate disease stage
    • Bone scan
    • Chest CT
    • Abdo CT
    • PET scan
207
Q

Medical Tx, Non-Hodgkin’s Lymphomas

(3)

A

Varies c histology and disease progression

  • Radiation therapy, if localized
    • In combo c chemotherapy if advanced
  • Chemotherapy
    • CHOP
    • Advanced cases only
  • Rituximab – binds to B cell receptors to stim. apoptosis
    • For relapse or refractory follicular lymphoma
208
Q

Classification, Non-Hodgkin’s Lymphoma

(“2”)

A

Classification system is still evolving. Existing systems

  1. Clinical features
  2. Pathologic features
209
Q

Prevelance, Non-Hodgkin’s Lymphoma vs. Hodgkin’s Lymphoma

A

Non-Hodgkin’s 3x more common than Hodgkin’s

210
Q

PET Scan

(definition, clinical use in hematology)

A

Definition: Imaging technique that evaluates tagged glucose and its position in the body. Helps evaluate physiology

Use: Diagnosis of Non-Hodgkin’s Lymphomas

211
Q

Define Multiple Myeloma

A

Disseminated malignant neoplasm of plasma cells that infiltrates BM

212
Q

Etiology, Multiple Myeloma

A
  • Male > female
  • Exact etiology unknown. Some hypotheses
    • Genetics
    • Autoimmune disease
    • Environmental toxins
    • Agricultural chemicals
      • Herbicides
      • Pesticides
213
Q

Pathophysiology, Multiple Myeloma

(7 steps, 8 end results)

A
  1. Malignant plasma cells invade BM
  2. Abnormal cells consume BM and progress to invade bone matrix (cortex)
  3. Formation of osteolytic lesions
  4. Simultaneous co-infiltrations due to wide distribution of plasma cells
  5. Plasma cells produce excessive immunoglobulins
  6. High circulating protein from immunoglobulins degrade kidney and bone
  7. Result in various complications
    • Dec. functional antibodies - infection
    • Protein overload in kidney
      • Pyelonephritis
      • Renal failure
      • Renal calculi (stones due to inc. calcium or uric acid)
    • Bone destruction
      • Hematologic imbalance (anemia, abnml WBC leukocytosis)
      • Fractures at osteolytic lesions
      • Hypercalcemia from bone degradation byproduct
      • Hyperuricemia from cell degradation products
214
Q

S/Sx, Multple Myeloma

(6 symptoms, 4 signs)

A

Symptoms

  • Pts typically present c complaints concerning
    • Anemia
    • Bone pn, most severe in
      • Back
      • Ribs
      • Hips
    • Infection
  • Fever
  • Malaise
  • Weight loss

Signs

  • Anemia s/sx
    • Pallor
  • Bone destruction
    • Osteoporosis
    • Pathologic fx
    • Vertebral compression fx
215
Q

Differential Dx, Multiple Myeloma

(6)

A
  1. Anemia
  2. MSK dysfunction
  3. Malignant lymphoma
  4. Metastatic carcinoma
  5. Monoclonal gammopathies or uncertain origin
216
Q

Diagnostic Tests, Multiple Myeloma

(6)

A
  1. X-ray – lytic lesions in skull, pelvis, spine
  2. CBC
    1. Moderate-severe anemia
    2. Rouleaux formation of RBC
  3. Bence Jones urinalysis – positive
  4. Electrolyte levels - Hypercalcemia
  5. Serum protein electrophoresis – hallmark test
    1. Monoclonal spike in beta or gamma region (“M spikes”)
    2. Note- can be spiked in other diseases, must go with other dx tests
  6. Bone marrow aspirate – inc. plasma cell precursors
217
Q

Medical Tx, Multiple Myeloma

(3)

A

Only with more progressive disease. May be monitored at first

  1. Chemotherapy to suppress plasma cell growth, thus dec. pn
    • VAD
    • TD
  2. Adjunctive radiation
  3. Dialysis if kidneys fail
218
Q

Surgical Tx, Multiple Myeloma

A

BM Transplant

219
Q

Patient Education, Multilple Myeloma

A
  1. Prognosis is improving. Currently 4-6 years c tx
  2. Avoid the following to prevent irritation
    • Heavy lifting
    • Dehyrating
220
Q

Platelets

(origin, function, normal count, normal lifespan)

A

Origin: fragment from megakaryocytes after prdcn stimulation via thrombopoietin (synth in liver)

Function: hemostasis

  • occlude small BV openings
  • contribute factor III to extrinsic clotting pathway

Count: 250,000-500,000/ml blood, although counts vary c geographic location and lab equipment

Lifespan:

  • formation - 5 days
  • half life - 8→12 days
221
Q

Platelet Disorders

(2 “categories”, general defs)

A
  1. Essential Thrombocytosis - inc. platelets
  2. Thrombocytopenia - dec. platelets
222
Q

Define Essential Thrombocytosis

A

Myeloproliferative disorder characterized by increased platelets with no other direct cause

223
Q

Etiology, Essential Thrombocytosis

(2)

A
  1. Median age 50-60 yo
  2. Correlation c JAK2 mutation (same mutation as CML)
224
Q

Pathophysiology, Essential Thrombocytosis

(2)

A
  1. Excessive platelet formation without trigger
  2. Abnormal and excess platelets c attempted removal by spleen
225
Q

S/Sx, Essential Thrombocytosis

(1 symptom, 3 signs)

A

Symptoms Increased bleeding

Signs

  1. Thrombosis in normal and abnormal venous clotting locations
    • Typical (for other clotting disorders) – DVT
    • Atypical (for other clotting disorders)
      • Mesenteric vein
      • Hepatic vein
      • Portal vein
  2. Bleeding, mucosal
  3. Splenomegaly
226
Q

Differential Dx, Essential Thrombocytosis

(5, 1 important one)

A
  1. Chronic myelogenous leukemia
  2. Agnogenic myeloid metaplasia c myelofibrosis
  3. Myelodysplastic syndrome
  4. Polycythemia vera
  5. Secondary thrombocytosis
227
Q

Diagnostic Tests, Essential Thrombocytosis

(3)

A
  1. CBC
    • Platelet count > 500,000/mcl (can be in the millions)
    • Large, abnormal platelets on peripheral smear
    • WBC count – slightly increased c increased blasts
  2. Bone marrow biopsy – increased megakaryocytes
  3. Genetic testing – No Philadelphia chromosome, unless this is early CML
228
Q

Medical Tx, Essential Thrombocytosis

(1)

A

Refer to hematologist, who will prescribe Hydroxyurea for myelosuppression

Requires regular monitoring via CBC

229
Q

Pt Education/Prevention, Essential Thrombocytosis

(2)

A
  1. 5% cases transform to leukemia, esp CML
  2. Prognosis, avg >15 years
230
Q

Clinical Pearls, Essential Thrombocytosis

(2)

A
  1. Test for Philadelphia chromosome to R/O CML
  2. If platelet count in the realm of 500,000 and there are no other s/sx you can have your pt return in a couple weeks to retest platelet counts
    • Decreased – continue to monitor
    • Increased/remained – refer to hematologist
231
Q

Thrombocytopenia

(definition, 3 general causes)

A

Definition: Decreased platelet count, c risk of spontaneous hemorrhage if plt count < 20,000/mcl

Causes:

  1. Decreased platelet production
  2. Increased platelet destruction
  3. Platelet dysfunction
232
Q

Causes of Dec. Platelet Production in Thrombocytopenia

(3)

A
  • Bone marrow failure
    • Congenital disorders
    • Acquired
      • Aplastic anemia
  • Bone marrow infiltration
    • Massive replacement of bone marrow by leukemic cells, myeloma, lymphoma
  • Radiation or chemotherapy exposure
233
Q

Platelet Destructive/Dysfunctional Thrombocytopenias

(list 5)

A
  • Immune Thrombocytpoenic Purpura
  • Thrombotic microangiopathies
    • Thrombotic Thrombocytopenic Purpura
    • Hemolytic Uremic Syndrome
  • Heparin Induced Thrombocytopenia
  • Disseminated Intravascular Coagulopathy
234
Q

Define Immune Thrombocytopenia Purpura (ITP)

A

Isolated thrombocytopenia secondary to anti-platelet antibodies

235
Q

Etiology, Immune Thrombocytopenia Purpura

A

Diagnosis of exclusion

  • Usually idiopathic
  • Associated c the following
    • Lupus
    • Lymphoma
    • Hep B
    • Hep C
    • HIV
    • Drugs
      • Sulfa
      • Thiazides (anti-convulsive)
      • Cimetidine
236
Q

S/Sx, Immune Thrombocytopenia Purpura (ITP)

(1 symptom, 3 signs within 1 category)

A

Symptoms Spontaneous bleeding

Signs Mucocutaneous bleeding, spontaneous when plt count < 20,000-30,000

  1. Bruising
  2. Nosebleeds
  3. Gingival bleed
237
Q

Pathophysiology, Immune Thrombocytopenia Purpura (ITP)

(3 steps)

A

a. Anti-platelet antibodies attach to normal platelets
b. Macrophages in liver and spleen phagocytize platelets
c. Resulting thrombocytopenia

238
Q

Differential Dx, Immune Thrombocytopenia Purpura (ITP)

A

Other destructive/dysfunctional platelet thrombocytopenias

  • Thrombotic microangiopathies
    • Thrombotic Thrombocytopenic Purpura
    • Hemolytic Uremic Syndrome
  • Heparin Induced Thrombocytopenia
  • Disseminated Intravascular Coagulopathy
239
Q

Diagnostic Tests, Immune Thrombocytopenia Purpura (ITP)

(3)

A
  1. CBC – Isolated thrombocytopenia
    1. Platelet Count
      1. Acute: 10,000 – 20,000/mcl
      2. Chronic: 25,000 – 75,000/mcl
    2. RBC studies – potential mild anemia
  2. Coagulation studies – normal
  3. Disease specific testing, varies upon etiology
    1. Hep B
    2. Hep C
    3. HIV
240
Q

Medical Tx, Immune Thrombocytopenia Purpura (ITP)

(6)

A

Refer to a hematologist **as soon as levels drop below normal. **He/she will

  1. Monitor, if chronic. Spontaneous resolution often occurs
  2. Steroids
  3. IV immunoglobulin
  4. Rituximab
  5. Splenectomy
  6. Treat underlying co-morbidity or eliminate offending agent
241
Q

Emergency Tx, Immune Thrombocytopenia Purpura (ITP)

A

Platelet transfusion for life threatening bleeding

242
Q

Rituximab

(MOA, role in ITP)

A

MOA: genetically engineered monoclonal antibody agnst CD20 ag on surface of normal and malignant B cells

Role in ITP: attack dysfunctional B cells to decrase severity of thrombobytopenia

243
Q

Thrombotic Microangiopathies

(definition, list 2)

A

Def: Potentially fatal thrombocytopenia due to incorporation of plts into thrombi in microvasculature. Forms tiny clots in circulatory system. Results in

  1. blockage of normal cell circulation
  2. lack of available platelets for necessary clotting

Diseases:

  1. Thrombotic Thrombocytopenic Purpura (TTP)
  2. Hemolytic Uremic Syndrome (HUS)
244
Q

Etiology, Thrombotic Thrombocytic Purpura

(6)

A

Know all of these

  1. Viruses (HIV)
  2. Bacteria
  3. Pregnancy
  4. Autoimmune
  5. Cancer
  6. Medications
    • Cyclosporine
    • Clopidogrel
245
Q

Pathophysiology, Thrombotic Thrombocytic Purpura

(5 steps)

A
  1. Antibodies against vonWillenbrand factor cleaving protease (ADAMTI-13)
  2. Multimer accumulation within vasculature
  3. Bridging of multimer
  4. Excessive plt aggregation
  5. Circulating clots prevent platelet use in necessary clots
246
Q

S/Sx, Trombotic Thrombocytic Purpura

(3 symptoms, 5 signs)

A

Symptoms Anemia symptoms, bleeding difficulties, DVT

Signs 25% of patients will have pentad:

  1. Thrombocytopenia
  2. Hemolytic anemia
  3. Fever
  4. Neuro defects (HA, somnolence, delirium, seizures)
  5. Renal insufficiency
247
Q

Differential Dx, Thrombotic Thrombocytic Purpura

(4)

A

Other thrombocytopenias

  • Immune thrombocytopenia purpura
  • Thrombotic microangiopathies
    • Hemolytic Uremic Syndrome
  • Heparin Induced Thrombocytopenia
  • Disseminated Intravascular Coagulopathy
248
Q

Diagnostic Tests, Thrombotic Thrombocytic Purpura

(6)

A
  1. CBC
    • Hemolytic anemia
      • Schistocytes s sickled cells
      • Normocytic, normochromic RBC
      • Elevated WBC count
    • Thrombocytopenia, platelets < 20,000
  2. LDH – increased
  3. Bilirubin (direct and indirect) – increased
  4. Reticulocyte count – increased
  5. Coagulation studies – normal
  6. BUN/Cr – increased only in very severe TTP
249
Q

Medical Tx, Thrombotic Thrombocytic Purpura

(3)

A
  1. Plastmapheresis
  2. Steroids
  3. IV immunoglobulin
250
Q

Surgical Tx, Thrombotic Thrombocytic Purpura

A

Splenectomy

251
Q

Pt Ed/Prevention, Thrombotic Thrombocytic Purpura

A

Prognosis - 5% s plasmapheresis

252
Q

Define Hemolytic Uremic Syndrome

A

Thrombocytopenia due to incorporation of plts into thrombi in microvasculature c associated RBC shearing in microcirulation

253
Q

Etology, Hemolytic Uremic Syndrome

(3 aspects, 2 major takeaways)

A
  • Predominantly pediatric population
  • Usually associated c current/recent diarrhea (usually due to food poisoning)
  • Typical causative agents
    • Toxigenic E. Coli (0157:H7)
    • Viruses (HIV)
    • Bacteria
    • Pregnancy
    • Autoimmune
    • Cancer
    • Medications
      • Cyclosporine
      • Clopidogrel
254
Q

Pathophysiology, Hemolytic Uremic Syndrome

(6 steps)

A
  1. Endothelial damage from toxigenic E Coli (0157:H7), cancer, HIV
  2. Circulating small platelet aggregations
  3. Extension of aggregates throughout vessel walls
  4. RBC lyse when passing through vessels, creating ineffective schystocytes
  5. Platelets unavailable for necessary clotting
  6. Renal insufficiency
255
Q

S/Sx, Hemolytic Uremic Syndrome

(3 symptoms, 5 signs)

A

Symptoms Anemia symptoms, bleeding difficulties, DVT

Signs 25% of patients will have pentad:

  1. Thrombocytopenia
  2. Hemolytic anemia
  3. Fever
  4. Neuro defects (HA, somnolence, delirium, seizures)
  5. Renal insufficiency
256
Q

Differential Dx, Hemolytic Uremic Syndrome

(4)

A

Other thrombocytopenias

  • Immune thrombocytopenia purpura
  • Thrombotic microangiopathies
    • Thrombotyc Thrombocytic Purpura
  • Heparin Induced Thrombocytopenia
  • Disseminated Intravascular Coagulopathy
257
Q

Diagnostic Tests, Hemolytic Uremic Syndrome

(6)

A
  1. CBC
    • Hemolytic anemia
      • Schistocytes s sickled cells
      • Normocytic, normochromic RBC
      • Elevated WBC count
    • Thrombocytopenia, platelets < 20,000
  2. LDH – increased
  3. Bilirubin (direct and indirect) – increased
  4. Reticulocyte count – increased
  5. Coagulation studies – normal
  6. BUN/Cr – increased
258
Q

Medical Tx, Hemolytic Uremic Syndrome

(4)

A
  1. Avoid antibiotics c E. Coli etiology, due to risk of resistance and increased toxin release from lysed cells
  2. Plastmapheresis
  3. Steroids
  4. IV immunoglobulin
259
Q

Surgical Tx, Hemolytic Uremic Syndrome

A

Splenectomy

260
Q

Pt Ed/Prevention, Hemolytic Uremic Syndrome

A

Prognosis – 5% s Plasmapheresis

261
Q

Clinial Pearls, Hemolytic Uremic Syndrome

(2)

A
  1. Avoid platelet transfusions – worsens thrombotic microangiopathy
  2. Vicious cycle – these pts need platelets because they are bleeding but platelets will advance the disease
262
Q

Define Heparin Induced Thrombocytopenia (HIT)

A

Decline in baseline platelet count of 50% or greater within 5-10 days of heparin exposure

263
Q

Etiology, Heparin Induced Thrombocytopenia (HIT)

(1)

A

Heparin patients

264
Q

Pathophysiology, Heparin Induced Thrombocytopenia (HIT)

(4 steps)

A
  1. IgG antibody formation against heparin platelet factor 4 (PF4) complexes
  2. Antigen-antibody complex formation and activation
  3. Macrophage phagocytosis, destroying the platelet
  4. Inability of BM to keep up c destruction, thus thrombocytopenia an dpro-thrombic state
265
Q

S/Sx, Heparin Induced Thrombocytopenia (HIT)

(1 symptom, 3 signs)

A

Symptoms Pain in the event of thrombosis

Signs

  1. Usually asymptomatic
  2. Thrombosis – 50% cases
  3. Bleeding is uncommon
266
Q

Differential Dx, Heparin Induced Thrombocytopenia (HIT)

(4)

A
  • Immune thrombocytopenia purpura
  • Thrombotic microangiopathies
    • Thrombotyc Thrombocytic Purpura
    • Hemolytic Uremic Syndrome
  • Disseminated Intravascular Coagulopathy
267
Q

Dx Studies, Heparin Induced Thrombocytopenia (HIT)

A
  1. CBC – significant thrombocytopenia, >50% decline from baseline
    • This is carefully monitored when heparin is initiated; often caught before s/sx occur
  2. Heparin PF4 antibodies – present only after thrombocytopenia (order after thrombosis)
268
Q

Medical Tx, Heparin Induced Thrombocytopenia (3)

A
  1. Discontinue heparin
  2. Anticoagulate c argatroban until platelet count >100,000
    • This is the threshold for safe Warfarin initiation, contraindicated otherwise
269
Q

Surgical Tx, Heparin Induced Thrombocytopenia

A

Limb amputation is thrombosis is significant and untreated

270
Q

Eergency Tx, Heparin Induced Thrombocytopenia

(1)

A

Initiate medical tx quickly as to avoid emboli

271
Q

Pt Eduacation/Prevention, Heparin Induced Thrombocytopenia (HIT)

(1)

A

Monitoring will be done by hospital staff any time heparin is initiated

272
Q

Define Disseminated Intravascular Coagulopathy (DIC)

A

Platelet consumptive syndrome characterized by simultaneous thrombosis and bleeding

273
Q

Etiology, Disseminated Intravascular Coagulopathy (DIC)

(6)

A

Severe underlying systemic illness

  • Sepsis
  • OB complications
  • Cancer
  • Severe transfusion rxn
  • Trauma
  • Burns
274
Q

Pathophysiology, Disseminated Intravascular Coagulopathy (DIC)

(4 steps)

A
  1. Systemic blood coagulation activation
  2. Fibrin generation and deposition
  3. Microvascular thrombi in various organs
  4. Consumption and subsequent exhaustion of coagulation PRO and platelets via microclot formation
275
Q

S/Sx, Disseminated Intravascular Coagulopathy (DIC)

(1 symptom, 3 signs)

A

Symptoms Extreme discomfort

Signs

  • Mucocutaneous bleeding at multiple sites (all orifices)
  • Shock
  • Digital ischemia leading to gangrene (depending on thrombosis location)
276
Q

Differential Dx, Disseminated Intravascular Coagulopathy (DIC)

(4)

A
  • Immune thrombocytopenia purpura
  • Thrombotic microangiopathies
    • Thrombotyc Thrombocytic Purpura
    • Hemolytic Uremic Syndrome
  • Heparin Induced Thrombocytopenia
277
Q

Diagnostic Tests, Disseminated Intravascular Coagulopathy (DIC)

(5)

A

All are vital for dx

  1. PT/INR – prolonged
  2. PTT – prolonged
  3. CBC
    • Thrombocytopenia
    • Hemolytic anemia
    • Schystocytes on peripheral smear
  4. D-Dimer assay – elevated
  5. Fibrinogen assay – decreased
278
Q

Medical Tx, Disseminated Intravascular Coagulopathy (DIC)

(4)

A

This is emergent

  1. Correct underlying problem
    • Antibiotics for sepsis
    • Surgery for trauma/burns
  2. Blood product in the presence of clinically significant hemorrhage
    • Goal = plt count 20,000 – 30,000
  3. Fresh Frozen Plasma (FFP) in patients c
    • Inc PT
    • Inc PTT
    • Significant bleeding
  4. Cryoprecipitate to replace fibrinogen
279
Q

Clinical Pearls, Disseminated Intravascular Coagulopathy

(3)

A
  1. Primary goal is to achieve hemodynamic balance in these pts
  2. Prognosis is very low
  3. Paradoxical condition; the tx will worsen the condition
280
Q

Define Hemophilia A

A

Genetic congenital Factor VIII deficiency, characterized by excessive clotting time

281
Q

Etiology, Hemophilia A

A

X-linked recessive trait therefore mostly males

282
Q

Pathophysiology, Hemophilia A

(3 steps)

A
  1. Lack of factor VIII interrupts the intrinsic pathway of the clotting cascade
  2. Platelets start to plug
  3. Definitive clot never forms
283
Q

S/Sx, Hemophilia A

(1 symptom, 1 sign c 5 common examples)

A

Symptoms Reports of spontaneous hemorrhage

Signs Hemorrhagic episodes s appropriate injury. Typical bleeds:

  1. Hemarthoses
  2. Epistaxis
  3. Intracranial bleeding
  4. Hematemesis
  5. Melana
284
Q

Differential Dx, Hemophilia A

(3)

A
  1. Hemophilia B
  2. vW Disease
  3. Vitamin K deficiency
285
Q

Dx Tests, Hemophilia A

(7)

A
  1. PTT – prolonged
  2. PT – normal
  3. Bleeding time – normal
  4. Fibrinogen – normal
  5. Platelets – normal
  6. Factor VIII Assay – decreased
  7. Factor IX Assay - normal
286
Q

Medical Tx, Hemophilia A

(2)

A
  1. Recombinant Factor VIII, periodic injections
  2. Desmopressin (DDAVP)
287
Q

Pt Education/Prevention, Hemophilia A

(2)

A
  1. Patients must be very careful and attentive c injuries. Example – every head injury warrants a CT
  2. Avoid aspirin; different MOA but reduced mechanisms for coagulation are always helpful
288
Q

Clinical Pearls, Hemophilias A and B

(2)

A
  • Only distinguishable factors between Hemophilias A and B are the CF assays and prevalence. Everything else is identical
  • Prevalence
    • Hemophilia A – 1/5,000 life male births
    • Hemophlia B - 1/25,000 male births
289
Q

Define Hemophilia B (Christmas Disease)

A

Genetic congenital Factor IX deficiency, characterized by excessive clotting time

290
Q

Etiology, Hemophilia B (Christmas Disease)

A

X-linked recessive trait therefore mostly males

291
Q

Pathophysiology, Hemophilia B (Christmas Disease)

(3 steps)

A
  1. Lack of factor IX interrupts the intrinsic pathway of the clotting cascade
  2. Platelets start to plug
  3. Definitive clot never forms
292
Q

S/Sx, Hemophilia B (Christmas Disease)

(1 sypmtom, 1 sign c 5 examples)

A

Symptoms Reports of spontaneous hemorrhage

Signs Hemorrhagic episodes s appropriate injury. Typical bleeds:

  1. Hemarthoses
  2. Epistaxis
  3. Intracranial bleeding
  4. Hematemesis
  5. Melana
293
Q

Differential Dx, Hemophilia B (Christmas Disease)

(3)

A
  1. Hemophilia B
  2. vW Disease
  3. Vitamin K deficiency
294
Q

Diagnostic Tests, Hemophilia B (Christmas Disease)

(7)

A
  1. PTT – prolonged
  2. PT – normal
  3. Bleeding time – normal
  4. Fibrinogen – normal
  5. Platelets – normal
  6. Factor VIII Assay – reduced
  7. Factor IX Assay - normal
295
Q

Medical Tx, Hemophilia B (Christmas Disease)

(2)

A
  1. Recombinant Factor IX, periodic injections
296
Q

Pt Education/Prevention, Hemophilia B (Christmas Disease)

(2)

A
  1. Patients must be very careful and attentive c injuries. Example – every head injury warrants a CT
  2. Avoid aspirin; different MOA but reduced mechanisms for coagulation are always helpful
297
Q

Define vonWillebrand Disease

A

Autosomal dominant congenital bleeding disorder, most common congenital coagulopathy

298
Q

Etiology, vonWillebrand Disease

A

Autosomal dominant disease

299
Q

Pathophysiology, vonWillebrand Disease

(2 steps)

A
  1. Origin depends on type
    • Type 1: Quantitative defect, not enough vWF production
    • Type 2: Qualitative defect, lack of vWF cofactor, ristocetin. While there is adequate vWF present it will not activate
  2. Without adequate functioning vWF, glycoprotein bridges cannot form in a coagulative event, preventing platelet plug formation and inhibiting effectiveness of the clotting cascade
300
Q

S/Sx, vonWillebrand Disease

(2 symptoms, 2 signs)

A

Symptoms

More predictable hemorrhage than hemophilia
Prolonged heavy menstrual bleeding

Signs

Mucosal bleeding
Skin bleeding/easy bruising

301
Q

Differential Dx, vonWillebrand Disease

(3)

A
  1. Hemophilia A
  2. Hemophilia B
  3. Vitamin K deficiency
302
Q

Diagnostic Tests, vonWillebrand Disease

(5)

A
  1. Bleeding time – prolonged
  2. PT – normal
  3. PTT – normal
  4. CBC – normal, including platelet count
  5. Specific assays depend on type
    • Type 1: decreased vonWillebrand factor assay
    • Type 2: decreased ristocetan assay
303
Q

Medical Tx, vonWillebrand Disease

(2)

A
  1. Desmopression (DDAVP, Stimate), PO or nasal spray
  2. vW concentrates, reserved for severe disaease or surgery
304
Q

Define Vitamin K Deficiency

A

Disruption in the clotting cascade due to deficient vitamin K

305
Q

Etiology, Vitamin K Deficiency

(3)

A
  1. Almost always due to decreased dietary intake of vitamin K
    • Dark leafy greens
    • Oils (soybean, cottonseed, canola, olive)
  2. Underlying liver disorder may also be a culprit – hepatitis, chronic alcoholism, etc.
  3. Post operative pts on broad spectrum bacteria that suppress colonic bacteria
306
Q

Pathophysiology, Vitamin K Deficiency

(1 c 4 aspects)

A

Inability to synthesize clotting factors vital cofactor in liver. Clotting factors affected:

II
VII
IX

X

307
Q

S/Sx, Vitamin K Deficiency

(1 sign, 1 symptom)

A

Symptoms Increased bleeding times

Signs Increased bleeding when encountered c offending agent

308
Q

Diagnostic Tests, Vitamin K Deficiency

(4)

A
  1. PT/INR – prolonged
  2. aPPT – normal
  3. Liver function test – elevated, esp c chronic alcoholism
  4. CF II, VII, IX, X assays – decreased
309
Q

Medical Tx, Vitamin K Deficiency

(2)

A
  1. Vitamin K (Phytonadion)
  2. Fresh frozen plasma if hemorrhaging
310
Q

Define Congential Venous Thrombosis (Group of Disease)

A

Abnormal clotting due to inheritable condition that leads to permanent hypercoaguable state if untreated

311
Q

Etiology, Congenital Venous Thrombosis Group

(5)

A

Not an exhaustive list, but these are rare genetic diseases pertaining to stoppage in hemorrhage. All contain positive family hx:

  1. Factor V Leiden (most common)
  2. Antithrombin III deficiency
  3. Protein S deficiency
  4. Protein C deficiency
  5. Antiphospholipid antibody syndrome
    • Anticardiolipin antibodies
    • Lupus anticoagulant
312
Q

Pathophysiology, Congenital Venous Thrombosis Group

(3 differnt options)

A
  • Factor V Leiden – mutated factor V cannot deactivate c Protein C
  • Antithrombin III/Pro S/Pro deficiency – vital ingredients for clotting cascade are absent
  • Antiphospholipid antibody syndromes – autoimmune attack on clotting ingredients
313
Q

S/Sx, Congenital Venous Thrombosis Group

(3 symptoms, 2 signs)

A

Symptoms

  1. Thrombotic event at young age s predisposition
  2. Recurrence within individual
  3. Extreme pn

Signs

  1. Erythema at clot location
  2. Swelling at clot location
314
Q

Differential Dx, Congenital Venous Thrombosis Group

(2)

A
  • Immune thrombocytopenia purpura
  • Thrombotic microangiopathies
    • Thrombotyc Thrombocytic Purpura
    • Hemolytic Uremic Syndrome
315
Q

Diagnostic Tests, Congenital Venous Thrombosis Group

(3)

A
  1. PTT – increased
  2. Factor assays, results vary c etiology
  3. US
316
Q

Medical Tx, Congenital Venous Thrombosis Group

(2)

A
  1. Anticoagulation c heparin, warfarin, and asprin (in heterozygous Factor V Leiden)
  2. Avoid hormone replacement therapy
317
Q

Clinical Pearl, Congenital Venous Thrombosis Group

A

These diseases can cause fetal demise. Genetic screening recommended for

General Medicine (4 decks)

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