Pathology Flashcards

1
Q

T/F: In severe illness such as a heart attack, pneumonia, etc, we should work the patient up for diabetes.

A

FALSE!!!!

Stress of illness often raises glucose to high levels. DO NOT work up for diabetes!

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2
Q

Bilirubin is a _____ function test.

A

liver

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3
Q

______ is derived from aging and circulating RBC’s, less so from ineffective erythropoiesis and the turnover of other heme‐rich cells (including hepatocytes)

A

Bilirubin

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4
Q

Increased indirect bilirubin may indicate what? (5)

A
  • MC: hemolysis
  • Common in newborns due to immature livers, hemolysis from ABO Ab
  • Sickle cell and spherocytosis pts (have mild lifetime jaundice)
  • Gilbert’s syndrome (fasting increases it more)
  • Rifampin
  • Trauma pts: reabsorpt. of hematoma; Medical pts: reabsorpt. of a hemorrhage w/in a lung infarct
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5
Q

These “liver function tests” reflect liver disease or obstruction / cholestasis rather than liver function. Interpret with caution and insight! What are they? (4)

A
  • AST
  • ALT
  • Alkaline phosphate
  • LDH
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6
Q

LDH (LD) is found in all tissues and increased values suggest nonspecific disease (ex: tumor marker for some germ cell cancers)
-HIGHEST LDH’s are in what? (2)

A
  • infectious mononucleosis

- megablastic anemias (intramedullay hemolysis)

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7
Q

What can affect the serum potassium? and therefore it is critical to draw the specimen properly and be aware of this when interpreting the results.

A

Any hemolysis!

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8
Q

When is the anion gap decreased?

A
  • multiple myeloma (cationic paraprotein)
  • hyponatremia, hypoalbunemia
  • bromide ingestion (anion)
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9
Q

What is synthesized in the liver by macrophages and fat cells in response to inflammation? What is it useful in determining?

A

High sensitivity C-reactive Protein

heart disease/cardiovascular risk

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10
Q

Leukocytes (WBC) polymorphonuclear cells (granulocytes) include what types of cells? (3)

A
  • neutrophils (segmented nucleus)
  • basophils (dark blue granules)
  • eosinophils (bilobed nuclei)
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11
Q

What do blasts (even just 1) indicate if seen in the peripheral blood?

A

Disease!!! (immature cells of undifferentiated lineage)

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12
Q

In adults, what is the reasonable range value for healthy WBC/mcL?

A

4500-11,000 WBC/mcL

17,000 for a three‐year old and 13,000 for a teen are reasonable upper limits

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13
Q

What are the two types of agranulocytes (mononuclear cells) considered WBC?

A
  • lymphocytes

- monocytes (macrophages outside the bloodstream)

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14
Q

TQ: A value of 8000 neutrophils/mcL is called…..

A

neutrophilia

an absolute neutrophil count above the top of the reference range

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15
Q

TQ: A value of 2500 neutrophils/mcL is called…..

A

Neutropenia

serious infection is likely only with counts below 1000/mcL and grave danger only with counts below 500/mcL

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16
Q

Neutrophilic bands are immature neutrophils. The presence of more than a few immature neutrophils in the peripheral blood is called what?

A

a left shift

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17
Q

Increased eosinophils (greater than ___/mcL) may be due to what?

A

600mcL

  • meds
  • parasitic infx
  • Asthma
  • Hodgkin’s dz
  • Churg-Strauss!!!
  • Addison’s
  • higher in afternoon due to circadian rhythym of cortisol
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18
Q

T/F: Pt’s with allergic or atopic disorders such as hay fever or other mild allergies don’t have elevated eosinophils

A

TRUE

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19
Q

What is the reasonable reference range of lymphocytes for adults?

A

800-4800/mcL

higher in children and teens

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20
Q

Activated lymphocytes such as atypical lymphocytes are found in what?
Depletion of CD4 cells is found in what?

A

infectious mononucleosis

HIV

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21
Q

Are WBC lymphocytes increased or decreased in whooping cough?

A

increased

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22
Q

What is the reference range for monocytes?

A

up to 900 mcL, 1200 for kids

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23
Q

What is the volume of RBC to volume of whole blood called?

A

Hematocrit

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24
Q

What are the reference ranges for hematocrit of an adult man and woman?

A

39-50% for men

35-46% for women

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25
Q

In what conditions would RBC count be decreased? (4)

A
  • anemia
  • microcytic normochromic
  • normochromic normocytic
  • macrocytic anemia.
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26
Q

In what conditions would RBC count be increased? (3)

A
  • polycythemia vera
  • secondary polycythemia
  • vigorous exercise.
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27
Q

At what mean cell volume do most hemolytic anemias fall?

A

80-100 (normal range…normocytic)

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28
Q

What is the mean cell volume for macrocytic diseases such as megaloblastic anemia?
Normocytic?
Microcytic (iron deficiency and thalassemias)?

A

> 100 macrocytic

80‐100 normal range, normocytic

< 80 microcytic

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29
Q

______ anemias lack hemoglobin. The red cell tends to be pale and small because the RBC precursor cannot make enough hemoglobin

A

Hypochromic

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30
Q

What are some examples of hypochromic anemias? (3)

A
  • Iron deficiency
  • Thalassemia
  • Severely defective porphyrin synthesis
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31
Q

What is increased in hereditary spherocytosis? (membrane fragments are lost so the hemoglbin is more dense and stains purply)

A

MCHC (Mean corpuscular hemoglobin concentration…a measure of the concentration of hemoglobin in a given volume of packed red blood cells)

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32
Q

RDW (red blood cell distrib) reference range is 11.5 to 14.5%. If the value is above this, what is suggested?

A

Anisocytosis (variation in size)

note: may indicate the degree of poikilocytosis (variation in shape)

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33
Q

________ count is an indication of the responsiveness of the bone marrow to decreased RBCs.

A

Reticulocyte

Reticulocytes are immature RBC’s or some times called shift cells. They are larger than normal (7 μm) RBC’s

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34
Q

What are the small fragments of megakaryocytes that are released into the blood called? What do they aid in?

A

Platelets: they aid in clotting since they contain activators of coagulation

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35
Q

What is thrombocytosis? What is it due to? (7)

A

increased platelet count due to reactive conditions such as:

  • infections
  • iron def
  • splenectomy
  • hypothermia
  • myeloproliferative disorders
  • leukemia
  • polycythemia vera
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36
Q

When is platelet count decreased? (6)

A
  • Incr/decreased destruction
  • pooling in spleen
  • immunologic mechanisms
  • drugs like heparin
  • dilutional coagulopathies
  • infection
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37
Q
  • May raise the RDW
  • Problem with cytoskeleton or hemoglobinization
  • Extreme variation in shape and size
  • May occur after a transfusion in a pt with microcytic anemia
A

Poikilocytosis

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38
Q
  • Measure of variation in RBC size
  • Just a marker that something’s wrong
  • May occur after a transfusion in a pt with microcytic anemia
A

Anisocytosis

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39
Q
  • Bizarrely-shaped red cells
  • Spectrin mutation
  • Autosomal recessive variant on hereditary spherocytosis
A

Hereditary pyropoikilocytosis

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40
Q
  • These cells lack the central pallor, are small, round, and slightly darker in color
  • Cytoskeleton may be defective or portions of the membrane may have been removed&raquo_space; weak, less deformable membrane
A

Spherocytes

“Hereditary Spherocytosis”

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41
Q

RBC acanthocytes are a famous marker for what?

A

abetalipoproteinemia

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42
Q
  • Coarse granulation resulting from aggregation of RNA
  • A non-specific finding in anemia
  • Classic example = lead poisoning
  • Seen in Beta-thalassemias
A

RBC basophilic stippling

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43
Q

Lead inhibits the activity of what 2 enzymes involved in heme synthesis?
How are ringed sideroblasts formed?

A

Delta aminolevulinic acid and ferrochelatase.

Inhibiting ferrochelatase&raquo_space; ringed sideroblasts
(iron remains within the mitochondria of the red cell precursors rather than joining the globin chains to make Hb molecules)

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44
Q

What are Howell-Jolly bodies?
If you see these, the pt may not have what organ?
What kind of anemia do you see Howell-Jolly bodies?

A
  • Howell-Jolly bodies are fragments of nuclear material (DNA) in RBCs.
  • Pt may not have a spleen, which typically removes them quickly.
  • Hereditary spherocytosis (normocytic anemia with predominantly extravascular hemolysis)
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45
Q

In a blood smear, how could tell which cell is “polychromatophilic?”

A

It will be appear more purple because it hasn’t lost all of its purple-staining RNA.

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46
Q
  • A spherocytosis variant

- Normal in camels, llamas, alpacas

A

Ovalocytes (elliptocytes)

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47
Q

Target cells. Think…

A

Hemoglobinopathy

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48
Q

Teardrop cells…

A

Something replacing the marrow.

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49
Q

Intracellular ring forms make the diagnosis of what?

A

Malaria

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50
Q
  • Intra- AND extracellular rings helps to differentiate this protozoan from malaria.
  • Use ticks as reservoir
A

Babesia

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51
Q
  • Elongated and pale cells

- Seen in true iron deficiency anemia

A

Pencil cells

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52
Q

Iron-laden mitochondria seen in what?

The mitochondria are termed what?

A

Sideroblastic anemia

iron-laden mitochondria = Pappenheimer bodies

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53
Q

Paroxysmal cold hemoglobinuria causes what to happen between RBCs?

A

Agglutination

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54
Q

Rouleaux formation (coin-stacking of RBCs) is suggestive of what?

A

Plasma cell dyscrasia / myeloma / paraproteinemia

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55
Q

96% of a normal adult’s Hb is:
3% of a normal adult’s Hb is:
1% of a normal adult’s Hb is:

A

(96%) HbA = 2A + 2B
(3%) HbA2 = 2A + 2D
(1%) HbF = 2A + 2G

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56
Q

What are the 4 alpha chain shortages seen in alpha-thal?

A

1 gene deleted: asymptomatic
2 genes deleted: cis = worse, risk of severe thal in offspring, Asians … trans = Africans
3 genes deleted: 4B = HbH
4 genes deleted: 4G = Hb Bart’s

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57
Q

What is the sequence of RBC precursors? (5)

A
  • Pronormoblast
  • Basophilic normoblast (prorubricyte)
  • Polychromatophilic normoblast (rubricyte)
  • Orthochromatophilic normoblast (metarubricyte)
  • Reticulocyte (“young reds”) – nucleus is exported before this step
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58
Q

How would you discriminate a pronormoblast from other RBC precursor cells in a blood smear?

A

Pronormoblast:

  • Large, circular cell housing a large, circular nucleus
  • Thin rim of cytoplasm around the nucleus
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59
Q

Describe how anemia presents clinically.

A

Anemia presents with signs and symptoms of hypoxia:

  • Weakness, fatigue, malaise, dyspnea
  • Pale conjunctiva and skin
  • Headache and lightheadedness
  • Angina, esp with preexisting CAD
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60
Q

What 3 things do you watch for when diagnosing hemolytic anemias?

A
  • Reticulocytosis
  • Incr indirect bilirubin
  • Incr LDH
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61
Q
  • Inherited defect of RBC cytoskeleton-membrane tethering proteins
  • Membrane blebs are formed and lost over time – loss of membrane renders cells round (“spherocytes”) instead of disc-shaped … spherocytes are less able to maneuver through splenic sinusoids and are consumed by splenic macrophages&raquo_space; anemia
A

Hereditary spherocytosis

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62
Q
  • Spherocytes with loss of central pallor
  • Incr RDW and incr MCHC (dark color)
  • Splenomegaly, jaundice, and incr risk for bilirubin gallstones (extravascular hemolysis)
  • Incr risk for aplastic crisis with parvovirus B19 infection of erythroid precursors
A

Hereditary spherocytosis

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63
Q

Hereditary spherocytosis is diagnosed with what test?

A

Osmotic fragility test – reveals increased spherocyte fragility in hypotonic solution

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64
Q

What are reticulocytes?

A

Young RBCs released from the bone marrow

-Identified on blood smear as larger cells with bluish cytoplasm (due to residual RNA)

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65
Q

What are Heinz bodies?

A deficiency in what enzyme will cause Heinz bodies to precipitate?

A

Denatured Hb that is often attached to the membrane

G6PD deficiency

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66
Q

How are Heinz bodies removed from RBCs?

What kind of cells result from their removal?

A

Heinz bodies are removed from RBCs by splenic macrophages, resulting in “bite cells.”

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67
Q
  • X-linked recessive disorder resulting in reduced half-life of G6PD; renders cells susceptible to oxidative stress
  • Oxidative stress (e.g., fava beans) precipitates Hb as Heinz bodies
  • Presents with hemoglobinuria and back pain hours after exposure to oxidative stress
A

G6PD deficiency

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68
Q
  • AR mutation in ß chain of Hb
  • Normal glutamic acid (hydrophilic) is replaced with valine (hydrophobic)
  • Gene carried by 10% of individuals of African descent (likely due to protective role against falciparum malaria)
  • Arises when 2 abnormal ß genes are present&raquo_space; >90% HbS in RBCs
A

Sickle cell disease

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69
Q

What happens to HbS when it gets deoxygenated?

What kind of cells does this result in?

A
  • HbS polymerizes when deoxygenated

- HbS polymers aggregate into needle-like structures&raquo_space; sickle cells

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70
Q

Which Hb prevents sickling?

A

HbF

-therefore children do not show symptoms for the first few months of life

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71
Q

What 3 things precipitate sickling?

A

Hypoxemia
Dehydration
Acidosis

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72
Q
  • Leg ulcers
  • Strokes
  • Retinal infarcts
  • Priapism
  • Painful bone infarcts
  • Thrombosis
  • Pain management
A

Sickle cell disease

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73
Q

You see autosplenectomy in what disease?

A

Sickle cell disease

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74
Q

Acute chest syndrome (vaso-occlusion in pulmonary vasculature) is the MC cause of death in adult pts of what disease?

A

Sickle cell disease

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75
Q

Massive erythroid hyperplasia ensues from sickle cell dz, leading to what 3 things?

A
  • Expansion of hematopoiesis into skull (‘crewcut’ appearance) and facial bones (‘chipmunk facies’)
  • Extramedullary hematopoiesis with hepatomegaly
  • Risk of aplastic crisis with parvovirus B19 infection of erythroid precursors
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76
Q

Explain sickle cell trait.

What does this result in?

A

One mutated and one normal ß chain; results in <50% HbS in RBCs, therefore these pts are generally asymptomatic

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77
Q

In sickle cell trait pts, sickling is caused by what 2 things?

A
  • Hypoxia (high altitude)

- Damaged renal medulla (electrolyte loss)

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78
Q
  • AR mutation in ß chain of Hb
  • Normal glutamic acid is replaced by lysine at 6th AA position of ß chain
  • Less common than sickle cell dz
  • Presents with mild anemia due to extravascular hemolysis
  • Characteristic HbC crystals (rod-shaped) are seen in RBCs on blood smear
  • Mostly target cells in the blood
A

Hemoglobin C disease

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79
Q
  • Result of the substitution of lysine for glutamic acid at the 26th amino acid of the ß globin chain (common in Asia)
  • Presents with mild microcytosis and target cells are seen
A

Hemoglobin E

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80
Q

4 ß chains are seen in what dz?

A
HbH disease
(alpha-thal when 3 genes are deleted)
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81
Q
  • Lots of little tiny red cells (MCV = 65 fL)
  • Mild anemia
  • Pancake cells
  • Maybe a few RBCs with basophilic stippling
A

Beta-thalassemia minor

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82
Q
  • Normocytic, normochromic anemia with predominant intravascular hemolysis
  • Acquired life-threatening dz characterized by complement-induced intravascular hemolytic anemia
  • Occurs episodically, often at night during sleep when pH is lowest (activates complement)
  • Main cause of death is thrombosis of the hepatic, portal, or cerebral veins
  • Iron deficiency anemia may result from losing Hb into the urine
A

Paroxysmal nocturnal hemoglobinuria (PNH)

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83
Q

___ infection causes sickness and death largely (but not entirely) by damaging the immune system, leading to opportunistic Infections and unusual tumors / odd cell proliferations

A

HIV infection

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84
Q

____ produces consolidation of the lungs. The alveoli fill with micro-organisms with a limited or absent inflammatory response.

A

PCP

Look like crushed ping-pong
Produces no sputum !!!

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85
Q

PCP in combination with ______ in MSMs = first identification of AIDS. Linked to low CD4 counts (below 200)

A

Kaposi’s

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86
Q

A high-resolution CT scan of “PCP” shows what?

A

Ground-glass opacities because the alveoli are either packed or empty.

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87
Q

How do you dx PCP? Hint: what test?

A

Blood test! 1-3-beta-d-glucan component of pneumocystis wall

May have to induce sputum to send to lab (biopsy, aspirate, etc)

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88
Q

_________ may be complication of HIV infx. Rates are growing due to homeless population and transmission from HIV pts.

A

Tuberculosis

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89
Q
  • A consumptive coagulopathy due to abnormal coagulation

- Poikilocytosis, schistocytes, decr platelets, microcytes

A

Disseminated intravascular coagulation (DIC)

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90
Q
  • Cancer association: Hodgkin, non-Hodgkin, thymomas
  • Systemic autoimmune dz – SLE, scleroderma, rheumatoid arthritis
  • Viral infections in children
  • Ulcerative colitis
  • Benign ovarian tumors, such as dermoid cysts
A

Hemolytic anemias

AHA warm

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91
Q
  • Cold agglutinins are due to IgM antibodies
  • May be assoc with a lymphoproliferative disorder
  • Can be 1) Idiopathic; 2) Secondary to infection, mycoplasma, mono, others; 3) Paroxysmal cold hemoglobinuria IgG against P blood groups
A

Hemolytic anemias

AHA cold

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92
Q

What is the test that detects whether Ig and/or complement is coating the surface of the pt’s RBCs?
What is a (+) test?

A

Direct antiglobulin test (“Direct Coombs” or DAT)

(+) test = agglutination of RBCs

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93
Q

What is the test that detects auto-antibodies or unexpected alloantibodies in pt’s plasma or serum?
What is a (+) test?

A

Indirect antiglobulin test (“Indirect Coombs”

(+) test = agglutination

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94
Q
  • An acquired disorder of nuclear maturation
  • High MCV, hypersegmented neutrophils, slight jaundice, high LDH
  • Deficiency of B12, folate, or both, which is essential for DNA synthesis
  • Pernicious anemia, inadequate intrinsic factor
  • PS: variable degrees of cytopenias, often pancytopenia
  • Hallmark feature = macrocytosis and oval macrocytes ***
  • RBC fragments, schistocytes and inclusions, such as Howell-Jolly bodies, basophilic stippling and Cabot rings
A

Megaloblastic anemia

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95
Q

Bone marrow findings in megaloblastic anemia:

A
  • Trilineage megaloblastic changes
  • Nuclear-cytoplasmic dyssynchrony
  • Erythroid hyperplasia
  • Granulocytic hyperplasia
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96
Q
  • A type of megaloblastic anemia in which the underlying cause is a defective intrinsic factor, which required for the absorption of vitamin B12
  • Atrophic gastric parietal cells (supposed to make IF)
  • Rare in children
A

Pernicious anemia

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97
Q
  • Low serum iron
  • High TIBC
  • Low % saturation
  • Low serum ferritin
  • Absent bone marrow iron ***
  • Microcytic, hypochromic and pencil cells
  • Pts like to chew ice cubes
  • Heavy periods, GI bleeding (hookworm), bad diet (junk food, vegans), malabsorption (famously sprue), achlorhydria
A

Iron deficiency anemia

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98
Q

27yo woman admitted with a hx of Crohn’s dz.

Labs: WBC 11.16, RBC 4.03, Hb 8.0, Hct 28.7, MCV 71.0, MCH 20.6, MCHC 28.6, RDW 17.2

A

Iron deficiency anemia

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99
Q

What is the test that detects whether Ig and/or complement is coating the surface of the pt’s RBCs?
What is a (+) test?

A

Direct antiglobulin test (“Direct Coombs”)

(+) test = agglutination of RBCs

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100
Q
  • Normocytic, normochromic morphology early
  • Microcytic, hypochromic morphology ***
  • Hypo-regenerating, normocytic/normochromic morphology later on
  • High ferritin
  • Low TIBC *
  • Low serum iron
  • Low % saturation
  • Interleukins acting on liver to produce hepcidin, which prevents marrow macrophages from releasing their iron to developing RBCs
  • Causes today: osteomyelitis, TB, RA, leprosy, CF, bedsores
A

Anemia of chronic inflammation (disease)

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101
Q
  • Selective decrease in bone marrow RBCs, without other associated cytopenias
  • PS: chronic normocytic to slightly macrocytic anemia
  • BM: normal cellular without erythroids
  • Incr EPO levels
  • Congenital causes, Diamond-Blackfan anemia
A

Pure red cell aplasia

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102
Q
  • Occasionally produces infection of the cervical lymph nodes in healthy people, especially children.
  • Can present as single-site lymphadenitis.
A

Mycobacterium avium organisms (like TB or other non-TB)

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103
Q
  • A common childhood viral infection (“fifth disease,” “erythema infectiosum, “slapped face”)
  • During illness, virus suppresses erythropoiesis
  • Cause of aplastic crisis seen in ppl with chronic hemolysis (spherocytosis, sickle cell disease)
A

Parvo B19

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104
Q

How do we diagnose CMV?

A
  • Rise in complement-fixing antibodies (4-fold titer over 2-4 weeks)
  • Finding IgM 30% or more of IgG.
  • Like the more common and more familiar Epstein-Barr infectious mononucleosis, CMV travels by kissing.
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105
Q
  • A family of genetic illnesses with a severe lack of erythroid precursors in the marrow from birth
  • Short stature, various craniofacial, thumb, and skeletal abnormalities
  • Incr HbF, EPO levels, RBC adenosine deaminase levels
  • RBC aplasia, decr reticulocyte count
  • Macrocytic anemia (MCV = 110-140)
A

Diamond-Blackfan anemia

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106
Q
  • In contrast to Diamond-Blackfan, these ppl have plenty of RBC precursors, but they do not develop properly
  • Illness is characterized by megaloblastic changes in the BM along with ineffective erythropoiesis (dyserythropoiesis) due to lack of maturation as a result of mitotic defects
  • Macrocytic anemia
  • Anisopoikilocytosis, basophilic stippling, low reticulocyte count, and Cabot rings
  • CDA II may mimic PNH due to incr hemolysis
A

Congenital dyserythropoietic anemia (CDA)

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107
Q
  • Toxoplasmosis is infection with the protozoan Toxoplasma gondii, which completes its life cycle in ___.
  • Avoid eating what?
A

cats (change the litter box!)

avoid eating undercooked meat!

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108
Q
  • An acquired disorder of nuclear maturation
  • Deficiency of B12, folate, or both, which is essential for DNA synthesis
  • Pernicious anemia, inadequate intrinsic factor
  • PS: variable degrees of cytopenias, often pancytopenia
  • Hallmark feature = macrocytosis and oval macrocytes ***
  • RBC fragments, schistocytes and inclusions, such as Howell-Jolly bodies, basophilic stippling and Cabot rings
A

Megaloblastic anemia

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109
Q

Causes of Mechanical Hemolysis (3)

A
  • Old-fashioned heart valves
  • Impact athletes (long-distance runners)
  • Karate chopping and bongo drumming
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110
Q
  • Pt who has Hb level above reference range and they’re not dehydrated
  • May be secondary to chronic hypoxia, high-affinity Hb’s, or anabolic steroid use by bodybuilders
A

Polycythemia

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111
Q

CMV in the colon was a common problem in which pts?

A

AIDS pts

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112
Q

T/F: Involvement of the brain in CMV infections is rare, but is devastating.

A

TRUE

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113
Q

If cryptococcosis is suspected, perform what first?

A

Neuroimaging

also look at CSF

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114
Q

__________ ________ was well-known, in the old days, as a sign that HIV infection was progressing to the late stage (“AIDS”).
-Usually, the CD4 count will be below 100.

A

Esophageal candidiasis

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115
Q

Cryptosporidiosis is infection by C. hominis or C. parvum.

-Protozoans live in what area of the body? What does this contribute to in AIDS pts?

A
  • Brush border of the small intestine

- Diarrhea in AIDS pts

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116
Q
  • Toxoplasmosis is infection with the protozoan Toxoplasma gondii, which completes its life cycle in ___.
  • Avoid eating what?
A

cats (change the litter box!)

avoid eating undercooked meat

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117
Q

How does toxoplasmosis present? How can we dx it?

A

-Primary infection is usually asymptomatic, or may mimic infectious mononucleosis

  • Find anti-toxoplasmosis IgM without IgG in the serum
  • Tachyzoites are found on fine-needle aspiration of the enlarged lymph nodes.
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118
Q

How does toxoplasmosis appear in the brain?

A

Ring-enhancing lesions (often in the basal ganglia)

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119
Q

Toxoplasmosis remains latent in the body after infection, and HIV patients who have antibodies as well as CD4 counts below ____ are prone to develop

A

100

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120
Q

Toxo is famous for involving the retina. What does it look like?

A

white fluff, less hemorrhage than CMV retinitis

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121
Q
  • Decr serum iron
  • Incr TIBC
  • Decr % saturation
  • Hypochromia and pencil cells
  • Pts like to chew ice cubes
  • Heavy periods, GI bleeding (hookworm), bad diet (junk food, vegans), malabsorption (famously sprue), achlorhydria
A

Iron deficiency anemia

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122
Q

27yo woman admitted with a hx of Crohn’s dz.

Labs: WBC 11.16, RBC 4.03, Hb 8.0, Hct 28.7, MCV 71.0, MCH 20.6, MCHC 28.6, RDW 17.2

A

Iron deficiency anemia

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123
Q

A single, narrow-based bud, and a capsule describes what?

Hint: Second most common cause of death in AIDS in Africa

A

Cryptococcus (C. neoformans)

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124
Q

Cryptococcus is related to which animal? Grows on which prep?

A

Pigeons!

India ink!

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125
Q

If cryptococcosis is suspected, perform what first?

A

Neuroimaging

also look at CSF

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126
Q
  • An unusual bacterial infection due to immune compromise
  • Solid masses of bugs
  • Looks like bumps on skin
A

Bacillary angiomatosis

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127
Q
  • Normocytic, normochromic morphology early
  • Microcytic, hypochromic morphology
  • Hypo-regenerating, normocytic/normochromic morphology later on
  • Interleukins acting on liver to produce hepcidin, which prevents marrow macrophages from releasing their iron to developing RBCs
  • Causes today: osteomyelitis, TB, RA, leprosy, CF, others
A

Anemia of chronic inflammation (disease)

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128
Q
  • Selective decrease in bone marrow RBCs, without other associated cytopenias
  • PS: chronic normocytic to slightly macrocytic anemia
  • BM: normal cellular without erythroids
  • Incr EPO levels
  • Congenital causes, Diamond-Blackfan anemia
A

Pure red cell aplasia

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129
Q

Progressive multifocal leukoencephalopathy (PML) involves which virus? What does it damage?

A
  • JC papovavirus
  • Damages the brain
  • CD4 count usually <200.
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130
Q

Cryptosporidiosis is infection by C. hominis or C. parvum. Protozoans live in what area of the body?

A

Brush border of the small intestine

diarrhea in AIDS

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131
Q

Today, the lab can test for cryptosporidium. Easiest is examining the _____ – cryptosporidia are acid-fast and can be easily seen on microscopy

A

stool

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132
Q

Does histoplasma capsulatum have a capsule?

A

NO!

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133
Q

Birds, bat droppings, starling roosts…think

A

histoplasmosis fungus
(2 micron yeasts) *

(It’s estimated that only about 1 in 2000 people who meet histoplasmosis in the wild will get seriously sick, but the risk is greater if there’s a big dose (camping in starling territory, cavers getting into bat guano)

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134
Q

Aspergillosis commonly follows which infection?

A

pneumocystis pneumonia

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135
Q

Fruiting body, 45 degree branching

A

Aspergillus

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136
Q

Spirochetosis of the colon seen in which infection?

A

Aspergillus

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137
Q

T/F: Zoster can occur at any CD4 count

A

True

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138
Q
  • Erythroviruses (______ __): usually produce a mild flu-like illness that may be followed with a rash and/or joint pain (“fifth disease” / “erythema infectiosum”).
  • Causes episodes of red cell production failure in folks with hemolytic anemias, or severe anemia before birth (“hydrops”)
A

parvo B19

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139
Q
  • An unusual bacterial infection due to immune compromise
  • Solid masses of bugs
  • Looks like bumps on skin
A

Bacillary angiomatosis

Bartonella species

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140
Q

What are 6 acquired causes of pure red cell aplasia?

A
  • Aplastic crisis
  • Parvovirus B19
  • Malnutrition
  • Drugs (chloramphenicol is infamous)
  • Thymoma or rarely other neoplasms
  • Idiopathic
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141
Q
  • A common childhood viral infection (“fifth disease,” “erythema infectiosum, “slapped face”)
  • During illness, virus suppresses erythropoiesis
  • Cause of aplastic crisis seen in ppl with chronic hemolysis (spherocytosis, sickle cell disease)
A

Parvo B19

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142
Q
  • Due to defective DNA repair mechanism. Marrow stem cells are lost.
  • Multiple congenital anomalies
  • Trilineage, bone marrow failure, usually at an early age ***
  • Substantial increased risk for other malignancies
A

Fanconi anemia

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143
Q
  • A family of genetic illnesses with a severe lack of erythroid precursors in the marrow from birth
  • Short stature, various craniofacial, thumb, and skeletal abnormalities
  • Incr HbF, EPO levels, RBC adenosine deaminase levels
  • RBC aplasia, decr reticulocyte count
  • Macrocytic anemia (MCV = 110-140)
A

Diamond-Blackfan anemia

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144
Q

_______ is dry or flaky scalp/skin. Likely to flare up as CD4 counts drop below 400, and become severe below 200.

A

Seborrhea

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145
Q

_____ ________– an Epstein-Barr virus effect in which the squamous epithelium on the tongue proliferates.

A

Hairy leukoplakia

146
Q

Outbreaks are generally water-borne, including swimming pools and ponds, and are more common where there’s poor sanitation, and during the rainy season in the tropics.

A

Cryptosporidium

147
Q
  • Thermally-dimorphic fungus – a mold in the soil, a yeast at 37 degrees
  • The yeasts multiply in the lungs
  • Before they can do any real damage, they are surrounded and either killed or walled off by macrophages.
A

Histoplasmosis

148
Q
  • In contrast to Diamond-Blackfan, these ppl have plenty of RBC precursors, but they do not develop properly
  • Illness is characterized by ineffective erythropoiesis (dyserythropoiesis) due to lack of maturation as a result of mitotic defects
  • Macrocytic anemia
  • Anisopoikilocytosis, basophilic stippling, low reticulocyte count, and Cabot rings
  • CDA II may mimic PNH due to incr hemolysis
A

Congenital dyserythropoietic anemia (CDA)

149
Q

Kaposi’s “sarcoma”, with pneumocystis pneumonia, led to the identification of AIDS and HIV

What causes it?
Where is characteristically located?
What does it look like under microscope?

A

Infection with herpes 8 (KSHV)

  • Dark purple skin/mouth nodules in a pt with AIDS
  • On chest/back, the palate and tip of nose

-Looks like granulation tissue (overgrowth of little blood vessels)

150
Q

-These are cancers of the lymphocytes that grow as solid masses.
-Many of them
are much more common in HIV patients than in other folks

A

Malignant lymphoma

151
Q

In the absence of EPO, the Hb level will drop to what?

A

around 8 gm/dL

152
Q
  • Present when the bone marrow is replaced by something that isn’t functioning marrow (TB, histoplasmosis, and cancer are the most familiar)
  • PS: nucleated RBCs, teardrop RBCs, young neuts
A

Myelophthisic process

153
Q

Causes of Mechanical Hemolysis (3)

A
  • Old-fashioned heart valves
  • Impact athletes (long-distance runners)
  • Karate chopping and bongo drumming
154
Q

Pt who has Hb level above reference range and they’re not dehydrated

A

Polycythemia

155
Q

The result of antigen binding to IgE on the surface of mast cells and basophils. These instantly de-granulate and release active substances into the surrounding tissue.

A

Type I hypersensitivity
“Anaphylactic”
“Immediate”

156
Q
  • Immediately released from mast cells

- Makes vessels dilate and leak

A

Histamine

157
Q
  • Immediate response

- Contract smooth muscle and make vessels leak

A

Leukotrienes

158
Q
  • Immediate response

- Causes bronchospasm and much mucus production

A

Prostaglandin D2

159
Q

The most important eosinophil attractant / activator is:

A

IL-5

160
Q

A marker for NK cells is:

A

CD57

161
Q
  • Red, itchy swellings of the skin that blanch easily on pressure
  • Often, but not always, about IgE / Type I immune injury
A

Urticaria

162
Q

Eosinophilic gastroenteritis.

Think…

A

Food allergy

163
Q

Often due to massive IgE-mediated response

A

Anaphylaxis

164
Q
  • Lowering the temperature causes mast cells to de-granulate

- Not immune-mediated

A

Cold urticaria

165
Q
  • Due to wet heat

- Not immune-mediated

A

“Cholinergic” urticaria

166
Q

Dendritic macrophages express IgE receptor (Fc[epsilon]RI) on their surfaces

A

Atopic dermatitis (aka eczema)

167
Q

Type of hypersensitivity in which Abs attach to Ags on the surfaces of a cell (fixed Ag), and then something injures or destroys the cell

A

Type II hypersensitivity

168
Q

Describe the Direct Coomb’s test.

A

Use Coomb’s reagent (Anti-IgG) to detect Ags present on the pt’s RBCs

(+) = agglutination

169
Q

Caused by Abs against the basement membranes of lung and kidney

A

Goodpasture’s disease

170
Q
  • Caused by Abs in the donor plasma against HLA Ags that the recipient has
  • Abs are often there because of a donor’s previous pregnancy… hence the strong preference for plasma donors being men
A

Transfusion-related acute lung injury

171
Q

MC cause of hypothyroidism from illness:

A

Autoimmunity

172
Q

Adrenal gland being destroyed by T cells.

Think…

A

Addison’s

173
Q
  • Islet of Langerhans being destroyed

- Both Abs and angry T-cells are generally present

A

Juvenile diabetes

174
Q
  • Abs cause hyperthyroidism by binding to and turning on the TSH receptors that drive thyroid hormone production
  • Abs cause proliferation of the loose connective tissue behind the eyes and on the shins
A

Graves disease

175
Q
  • Involves deposition of complexes of a soluble Ag and Abs, generally in vessel walls
  • These syndromes produce vasculitis (including possible infarcts), arthritis and/or glomerulonephritis
A

Type III hypersensitivity

176
Q

Explain the Arthus reaction.

A

This is the model for localized immune complex-mediated tissue injury.
Antigenic sensitization&raquo_space;
Ab formation&raquo_space;
Ag challenge&raquo_space;
Immune complex formation and inflammation

177
Q

Explain serum sickness.

A

Antigenic sensitization&raquo_space;
Ab formation&raquo_space;
Re-exposure to Ag&raquo_space;
Immune complex formation and inflammation

178
Q
  • “Most hated of all Type III immune-mediated reactions”
  • Kidney may turn white and bloodless before surgeon’s eyes as the vessels plug with complexes and fibrin … IgG precipitates in glomeruli and arterioles
A

Hyperacute rejection of the donor kidney

179
Q
  • In this type of hypersensitivity, special T-helper cells (T(D)) programmed to recognize a particular “altered self” Ag are stimulated
  • Great for ridding body of virally infected cells, cells harboring intracellular parasites (TB, some fungi), and perhaps tumor cells
  • Also the way the body acute-rejects transplanted organs
  • T-cell mediated injury
A

Type IV hypersensitivity

180
Q

What is anergy?

What does no response to an anergy test mean?

A

The lack of T(D) cell function.

No response to anergy test = T-helper cell problem! Think sarcoidosis, lymphomas or other cancers, HIV infection, measles, or really bad disseminated infections.

181
Q

What is the hapten in poison ivy?

A

Urushiol

182
Q

What is a patch test used to confirm?

A

T-cell hypersensitivity (Type IV)

183
Q

What is a halo nevus?

A

T-cells destroy melanocytes near a regressing nevus

184
Q

Granuloma formation is often done by what kind of cells?

A

Th1 cells, no Ab required

185
Q

TQ

Psoriasis rheumatoid arthritis, multiple sclerosis, Crohn’s, ulcerative colitis, and sarcoid are examples of what?

A

T-cell mediated hypersensitivity (Th1 and/or Th17)

186
Q

Acute cellular rejection occurs because of what type of cells?

A

CD8 cells

187
Q

Acute humoral rejection occurs because of what?

A

Antibodies

188
Q
  • Type III immune injury (type II has already occurred)
  • When pt gets an allograft and already has Abs against it
  • IgG precipitates in gloms and arterioles
  • Fibrinoid in the arterial walls
  • Kidney blanched and ruined in minutes
A

Hyperacute rejection

189
Q
  • Rejection mediated by T-cells

- See “tubulitis” and “endothelialitis”

A

Acute cellular rejection

190
Q
  • Caused by Abs, with both Type II and Type III injury
  • Blood vessel intima takes most severe injury, and we see edema, fibroblast proliferation, myxoid change, and accumulations of foamy macrophages (“graft arteriosclerosis”)
  • Look for C4d in the capillaries *
A

Acute humoral rejection

191
Q

TQ

  • Usually seen in old allografts
  • Vessels are narrowed or totaled
  • Intima is most thickened and (unlike atherosclerosis) it tends to be concentric *
  • T-cells do cause thickening of intima / media
A

Chronic transplant rejection

192
Q

TQ

  • Days to weeks after transplant
  • Often starts on the palms *
  • Generalized rash, sometimes loss of epidermis
  • Jaundice from damage to bile ducts
  • Ulcers along GI tract, may bleed
  • You’ll only see a few donor lymphocytes, mostly CD8’s
A

Acute graft-vs-host disease

193
Q
  • Appears later (after 100 days), more fibrosis and less obvious necrosis
  • Fibrosis of the dermis with loss of skin adnexal structures (resembles scleroderma)
  • Cholestatic jaundice from damage to bile ductules
  • GI damage with possible malabsorption and/or esophageal stricture
  • Damage to host T-cells, with immune compromise
  • CMV pneumonitis is the most feared infection
A

Chronic graft-vs-host disease

194
Q

Defective Fas or FasL (apoptosis triggers) leads to a:

A

Lupus-like illness

195
Q

The most-often-mutated gene in autoimmunity is:

A

PTPN22, a tyrosine kinase involved in lymphocyte responses.

196
Q
  • Immune system attacks the invader and the immune response then cross-reacts with something in the healthy tissues that resembles the microbe antigenically
  • E.g., strep cross-reacting with heart – rheumatic fever
A

Molecular mimicry

197
Q
  • Everyone keeps a few of their mother’s cells
  • All mothers keep a few of the cells of the children they have carried
  • It is rumored that the cells are more abundant in people with autoimmune disease

This is called:

A

Microchimerism

198
Q

Anti-nuclear Abs: Rim pattern.

Think…

A

Classic systemic lupus

Anti-dsDNA **

199
Q

Anti-nuclear Abs: Homogeneous pattern.

Think…

A

Drug-induced lupus

200
Q

Anti-nuclear Abs: Centromere pattern.

Think…

A

CREST syndrome

201
Q

Anti-nuclear Abs: Nucleolar pattern.

Think…

A

Scleroderma

202
Q
  • Anti-dsDNA
  • Anti-Sm (Smith)
  • Rim pattern on ANA
A

Systemic lupus

203
Q
  • Anti-ssDNA

- Anti-histone

A

Drug-induced lupus

204
Q

Anti-Ro/SSA (Sjogren’s A)

A

Neonatal lupus (heart block)

205
Q

Anti-La/SSB (Sjogren’s B)

A

Most Sjogren’s pts, and many other folks

206
Q

Anti-U1RNP (U1 ribonucleoprotein)

A

Mixed connective tissue disease (MCTD)

207
Q
  • Anti-Scl70 (anti-topoisomerase)

- Anti-RNA polymerase III

A

Scleroderma

208
Q

Anti-centromere

A

CREST, the “milder version of scleroderma”

209
Q

Anti-Jo (Jo1, transfer RNA synthetase)

A

Polymyositis / dermatomyositis

210
Q

Anti-Mi2 (against a helicase that remodels nucleosomes)

A

Dermatomyositis, esp when the rash is present

211
Q

Anti-SRP (signal recognizing polypeptide)

A

Polymyositis variant with severe necrotizing myopathy

212
Q
  • Anti-P155/TIF1(gamma)

- Anti-P140/MJ

A

Juvenile and paraneoplastic dermatomyositis

213
Q

Must have 4 of these 11:

  • Butterfly (malar) rash
  • Discoid rash
  • Anti-dsDNA autoAbs
  • UV light triggers flare-ups (photosensitivity)
  • Oral ulcers
  • Arthritis
  • Serositis
  • Renal disorder
  • Neuro disorder
  • Hematologic disorder
  • Type II and Type III hypersensitivity (immune disorder)
A

Lupus

214
Q

-Hematoxylin bodies
-Adventitial onion-skinning
-Immune complex deposits in a glomerulus (wire loop appearance)
Think…

A

Lupus

215
Q

A stripped nucleus opsonized by anti-nuclear Abs and now being devoured by phagocytes

A

LE cell

Think lupus.

216
Q

What is the lupus band test?

A

Granular immunoglobulin and complement @ dermal-epidermal junction.

217
Q

Mouth ulcers in lupus are due to:

A

Infarcts from vasculitis

218
Q
  • Sterile thrombi on the lines of heart valve closure

- May involve all surfaces of the leaflets

A

Libman-Sacks endocarditis in lupus

219
Q

Many lupus pts are killed by:

A

Deep vein thrombi / pulmonary emboli

220
Q
  • Photosensitivity
  • Atrophy and scaling, esp in the center of lesions
  • Hyperkeratosis, hydropic change, lymphocytes
A

Discoid lupus

221
Q

Which 3 drugs famously cause lupus?

A

Hydralazine
Procainamide
Isoniazid

222
Q

The salivary and lacrimal glands fill with very angry lymphocytes.

  • Dry eyes
  • Dry mouth
A

Sjogren’s syndrome

223
Q

What is the most feared complication of Sjogren’s?

A

Malignant lymphoma

224
Q
  • Autoimmune dz characterized by transient inflammation followed by dense fibrosis
  • Intimal layers of small blood vessels often undergo fibrous thickening
  • Almost always starts with Raynaud’s (intimal thickening in finger artery)
  • Sclerodactyly
  • Calcium deposits
  • Telangiectasias at nail bases or on face
  • Pulmonary fibrosis
  • Initial abundance of CD4+ T-cells
  • Dense dermis, flattened dermal-epidermal junction, lost adnexal structures
A

Scleroderma

“Systemic sclerosis”

225
Q

“Limited scleroderma” is known as:

A

CREST syndrome

  • Calcinosis (calcium deposits in skin)
  • Raynaud’s
  • Esophageal fibrosis
  • Sclerodactyly
  • Telangiectasias
226
Q
  • Scleroderma-like changes in a patch

- Initial CD4+ T-cell-rich inflammation, then fibrosis and vessel changes (like scleroderma)

A

Morphea

227
Q
  • Variant of scleroderma
  • Scar contracts over 1/2 of face
  • Deformation of underlying skull
A

Parry-Romberg

228
Q
  • Tight skin with many eosinophils in the subcutis

- Remits after glucocorticoid tx

A

Eosinophilic fasciitis

229
Q
  • Onset of weakness in proximal muscles: “I have trouble climbing stairs.”
  • Creatinine kinase and aldolase levels will be extremely high
  • Muscle and skin can calcify
  • Inflammatory arthritis and/or lung damage (usually interstitial fibrosis)
  • Upper esophagus involvement – swallowing may be a problem
  • Sometimes heart is involved
A

Polymyositis and Dermatomyositis

230
Q

What is the difference between polymyositis and dermatomyositis?

A

Dermatomyositis has the characteristic rash (heliotrope color – light purple)!

231
Q

In polymyositis, the distinctive lesion is extensive infiltration of muscle by:

A

CD8+ T-cells

232
Q

In dermatomyositis, the lymphocytic infiltrate is within or near the perimysium and around the blood vessels.
The infiltrate is a mix of what 3 cells?
What membrane attack complex is often seen in vessel walls (unlike polymyositis)?

A

CD4+ T-cells
B-cells
Plasma cells

Membrane attack complex = C5b-9

233
Q

In dermatomyositis, where are the muscle fibers most atrophied?

A

At the edges of the fascicles

234
Q

Gottron’s sign on the knuckles is a marker for:

A

Dermatomyositis

235
Q

“Mechanic’s hands”

Think…

A

Polymyositis / Dermatomyositis / Anti-synthetase (Jo1) syndrome

236
Q
  • This illness is the best-known of the “overlap” syndromes “combining features of lupus, scleroderma, RA, and polymyositis.”
  • Anti-U1-RNP Abs
  • Usually starts as synovitis of the fingers + Raynaud’s
  • Sclerodactyly may occur as in scleroderma
  • Skin changes range from rash to calcification
  • Good response to glucocorticoids and kidneys are spared!
  • Aggressive dz, death is usually due to pulmonary vascular sclerosis (“pulmonary HTN”) / pulmonary fibrosis
A

Mixed connective tissue disease (MCTD)

237
Q

Characteristic features:

  • Fibrosis with a “cartwheel” pattern
  • Plasma cells making IgG4 in the lesion(s)
  • Increased IgG4 in the blood (2/3 of pts)
A

IgG4-related disease

238
Q

3 tip-offs to vasculitis:

A
  • Mononeuritis monoplex
  • Palpable purpura
  • Anybody who “you just can’t tell why they are sick”
239
Q
  • “Migraine”
  • Vague aches and pains
  • Liver infarcts
  • Hep B/C and cryoglobulins as etiologies
  • Stroke and ballooning out of arteries (aneurysm)
  • Heart attack
  • Bowel infarcts
  • HTN
  • Gangrene
  • Peripheral nerve damage
  • Lungs spared!
  • Usually no hematuria
  • Patchy inflammation of all 3 layers of larger arteries; veins are spared! Fibrinoid change.
  • ANCA(–)
A

Polyarteritis nodosa, large-vessel type

240
Q
  • Vague aches and pains
  • Hemoptysis and infiltrates (lungs involved!) *
  • Stroke
  • Heart attack
  • Bowel infarcts
  • Nephritis / kidney failure
  • Gangrene
  • Peripheral nerve damage
  • Hematuria
  • Smaller veins and arteries show patchy 3-layer inflammation
  • p-ANCA(+) ** – positive anti-neutrophil cytoplasm test
A

Microscopic polyangiitis

small-vessel polyarteritis

241
Q
  • Caused by auto-Abs against proteinase 3 **
  • Granulomas and patchy necrosis in arteries and veins
  • Destruction of the face
  • Episcleritis, “saddle nose” *
  • Lung cavities and bleeds
  • Permanent kidney damage
  • Gangrene
  • c-ANCA(+) **
A

Wegener’s granulomatosis

242
Q
  • Purpura and IgA nephropathy
  • Arthritis
  • GI bleeding
  • Mild in children, severe in adults
A

Henoch-Schonlein purpura

243
Q
  • Marginally soluble protein precipitate in the cooler places and/or where blood is concentrated (i.e., kidney)
  • Another cause of vasculitis
A

Cryoglobulinemia

244
Q
  • Hallmark = abundant eosinophils in the peripheral blood and asthma
  • Mononeuritis multiplex very common
  • Vasculitis appears later; eosinophils abundant in vessel lesions
  • Often kills by occluding the microvasculature of the heart
  • Usually ANCA(+)
A

Churg-Strauss
(“Allergic granulomatosis”)
(“Eosinophilic granulomatosis with polyangiitis”)

245
Q
  • Autoimmunity against heat shock proteins
  • HLA-B51 ***
  • Mouth ulcers (always) **
  • Eye lesions
  • Genital ulcers
  • Skin lesions
  • Neurologic symptom(s)
  • Infarcts of anything
  • Thrombosis of anything
  • Amyloidosis
  • “Pathergy” – 48hr after a needlestick, pt develops an ulcer or blister ***
  • Affects arteries and veins of all sizes
A

Behcet’s dz

246
Q

Amyloid is ____-_______ anything.

A

Amyloid is BETA-PLEATED anything.

247
Q

Congo red with “apple green birefringence”

A

Amyloid

248
Q
  • Prolonged inflammation with macrophages

- Osteomyelitis, TB, leprosy, rheumatoid arthritis, etc.

A

Serum amyloid associated protein: Amyloid AA

249
Q

Plasma cell neoplasia, not necessarily obvious

A

Immunoglobulin light chains: Amyloid AL

250
Q

The bad forms have a mutation – may get a liver transplant

A

Transthyretin: Amyloid ATTR (AF)

251
Q
  • HLA light chains

- Longstanding kidney failure

A

ß-2 microglobulin: Amyloid ß2m (AH)

252
Q

Heart with restrictive cardiomyopathy (stiff heart) may be due to:

A

Amyloid

253
Q

Lardaceous spleen from amyloid in is the red or white area of the spleen?

A

Lardaceous spleen – WHITE area

254
Q

One-organ amyloidosis hits where?

A

Tongue

255
Q
  • Best-known defect in phagolysosome formation and function ***
  • Neutropenia
  • Giant granules in leukocytes
  • Defective primary hemostasis
  • Albinism ***
  • Peripheral neuropathy *
A

Chediak-Higashi disease

256
Q

Deficiencies of C2, C4, and especially Cq1 mimic:

A

Lupus

257
Q

C3 deficiency causes serious problems with: (2)

A
  • Bacteria

- Membranoproliferative glomerulonephritis

258
Q

Deficiency in C5, C6, C7, C8, or C9 causes difficulty dealing with:

A

Bacteria, esp Neisseria

259
Q

C1-esterase inhibitor deficiency produces:

A

Hereditary angioedema

-MC complement protein deficiency

260
Q

Candida (“yeast”), later CMV, pneumocystis, etc. all indicate problems with:

A

T-cells

261
Q

Bacterial infections once Mom’s Abs clear indicates problems with:

A

B-cells

262
Q

Baby has left arm paralyzed after receiving oral polio vaccine.
Think…

A

Severe combined immunodeficiency (SCID)

263
Q

Adenosine deaminase deficiency causes:

A

Severe combined immunodeficiency (SCID)

264
Q
  • Caused by lack of Bruton’s tyrosine kinase (BTK), which lets B-cells mature
  • Bacterial, giardia, and enterovirus infections around 6 months of age
  • Must avoid live polio and MMR vaccines
A

X-linked agammaglobulinemia

“Bruton’s”

265
Q
  • Lack of parathyroids
  • Lack of thymus
  • Midline cardiac defect(s) *
  • Hypocalcemia ***
A

DiGeorge syndrome / thymic dysembryogenesis

266
Q
  • Pts are unable to switch over from making IgM to making IgG, IgA, and IgE **
  • Most often involves mutated CD40L gene **
A

Hyper-IgM

267
Q
  • Many diseases, low Ig’s

- Lots of lymphoid tissue, with poor germinal centers

A

Common variable immunodeficiency (CVID)

268
Q

You may never know you have it until your second blood transfusion. Anaphylaxis occurs and workup reveals that you’re allergic to the donor’s IgA.

A

Isolated IgA deficiency

269
Q
  • Mutant SH2D1A signal transduction protein is defective

- Allows Epstein-Barr virus to produce a deadly lymphoma-like overgrowth of lymphocytes, or destroy liver and/or marrow.

A

Sex-linked lymphoproliferative syndrome

270
Q
  • X-linked
  • Tiny platelets
  • Eczema
  • Deficient T cells
  • WASp locus
A

Wiskott-Aldrich

271
Q
  • At least three illnesses with lots of IgE and too little of other Abs
  • Faulty neutrophil movement
  • Most troublesome bacteria = Staph
A

Hyper-IgE

“Job’s syndrome”

272
Q
  • Fungus that grows in the US Southwest and usually causes a mild lung infection when first encountered
  • A sphere full of yeasts (gumball machine) or hyphae with barrel-shaped spores may be seen in tissue during infections
A
Coccidioides immitis
("Coccidioidomycosis")
273
Q
  • “My worst case of the flu ever”
  • Sore throat, muscle aches, and otherwise resembling infectious mononucleosis
  • A rash and long duration may be tip-offs that it’s actually:
A

Acute retroviral syndrome

274
Q

Basophilia (4-5 basophils on high power field). Think…

A

Chronic Myelogenous Leukemia

CML

275
Q

Basophilia is characteristic of: (4)

A

Chicken pox
Small pox
Nephrotic syndrome
Basophilic leukemia (CML)

276
Q
  • WBC count > 50,000 uL
  • PS shows left shift (neutrophilic precursors in circulation)
  • Differentiated from leukemia
  • In non-leukemic neutrophilia, the LAP score rises with the WBC count
A

Leukemoid reaction

277
Q

4 causes of leukemoid reaction:

A
  • Severe sepsis
  • Burns
  • Tissue necrosis
  • G-CSF and GM-CSF therapy
278
Q

Toxic granulation is due to: (5)

What else can you see in these conditions?

A
  • Cytokine release
  • GSF
  • Severe infection
  • Trauma
  • Burns

Toxic vacuoles and Dohle bodies can be seen

279
Q
  • AR dz assoc with several different types of mucopolysaccharide disorders
  • An intense azurophilic granulation of the neutrophil cytoplasm
A

Alder-Reilly

280
Q

TQ

  • AR disorder characterized by giant cytoplasmic granules
  • Affects all granulated cells and lymphocytes
  • Assoc with partial albinism
A

Chediak-Higashi syndrome

281
Q
  • Multiple blue-gray inclusions of variable size and shape in the cytoplasm of WBCs
  • Composed of endoplasmic reticulum
  • In May-Hegglin anomaly, giant platelets are also seen
A

Dohle bodies

282
Q

Drug effects of G-CSF on PS and BM:

A

PS: toxic neuts, left shift, variable nuclear cytoplasmic dyssynchrony

BM: hypercellularity primarily in the granulocytes

283
Q

Drug effects of EPO on PS and BM:

A

PS: increased reticulocytes and occasional nucleated RBCs

BM: hypercellular marrow with increased erythroids and their precursors

284
Q

CD1:

A

Thymocytes and Langerhans cells

285
Q

CD3:

A

Thymocytes and mature T-cells

286
Q

CD4:

A

Helper T-cells, subset of thymocytes

287
Q

CD5:

A

T-cells and a small subset of B-cells

288
Q

CD8:

A

Cytotoxic T-cells, subset of thymocytes and some NK cells

289
Q

CD10:

A

Pre-B cells and germinal center B-cells

290
Q

CD19:

A

Pre-B cells and mature B-cells

291
Q

CD20:

A

Pre-B cells after CD19 and mature B-cells

292
Q

CD21:

A

EBV receptor; mature B-cells and follicular dendritic cells

293
Q

CD23:

A

Activated mature B-cells

294
Q

Progenitor cells have what cluster designation?

A

CD34

295
Q

CD30:

A

Activation marker

296
Q

All leukocytes have what cluster designation?

A

CD45

297
Q
  • Children and adolescents, episodic febrile illness, spontaneously regresses
  • Polyclonal hypergammaglobulinemia, massive LAD, effaced follicles, dilated sinuses, proliferation of histiocytes
  • No mitosis or necrosis
A

Rosai-Dorfman dz

298
Q

3 variants:

  • Hyaline-vascular type: young pts, lollipops and onion-skinning, plasmacytoid monocytes
  • Plasma cell variant: older pts, anemia, thrombocytopenia, incr ESR, hyperplastic lymphoid follicles, interfollicular plasma cells with preserved sinuses
  • Multicentric variant: older pts, severe systemic symptoms, anemia, thrombocytopenia, incr ESR, IL-6, LDH, and C-reactive protein
  • All variants have polyclonal hypergammaglobulinemia
  • Assoc w/ POEMS, Kaposi’s sarcoma, HIV
  • Hepatosplenomegaly
A

Castleman’s dz

299
Q
  • EBV (HHV-4) infection resulting in lymphocytic leukocytosis with reactive CD8+ T cells (Downey cells)
  • Generalized LAD
  • Pharyngitis
  • Splenomegaly
  • Monospot test used for screening – detects IgM that cross-react with horse or sheep RBCs
  • Follicular hyperplasia, expanded paracortex, RS-like cells, dilated sinuses, foci of necrosis
A

Infectious mononucleosis (IM)

300
Q
  • Reactive paracortical hyperplasia of the LN
  • Incr DCs, Langerhans cells, and histiocytes with melanin deposits and lipid vacuoles
  • Often assoc w/ benign or malignant skin dz’s. Follicular hyperplasia initially.
  • S100+ and CD1a–
A

Dermatopathic lymphadenopathy

301
Q
  • Diarrhea, wasting illness
  • LAD in multiple sites, foamy histiocytes in the sinuses and lymphoid areas
  • Caused by Tropheryma whipplei, a PAS+ bacilli with trilaminar walls
A

Whipple’s lymphadenitis

302
Q
  • A necrotizing, granulomatous lymphadenitis caused by Bartonella henselae
  • U/L LAD, matted lymph nodes with necrotizing granulomas, central microabscesses with neuts, palisading epithelioid cells, and clusters of pleomorphic bacilli
  • B. henselae is (+) for silver stains and gram stains, or IHC
A

Cat scratch dz

303
Q
  • Intermediate-sized B cells (CD20); assoc w/ EBV *
  • Classically presents in a child or young adult (Afro American form = jaw; sporadic form = abdomen)
  • Translocation of MYC (Chr. 8) – t(8;14) is MC
  • Characterized by numerous mitotic figures and “starry-sky” appearance on microscopy
A

Burkitt lymphoma

304
Q
  • Large B-cells (CD20) that grow diffusely in sheets
  • MC form of non-Hodgkin lymphoma
  • Clinically aggressive (high-grade)
  • Presents in late adulthood as enlarging LN or extranodal mass
  • Arises sporadically or from transformation of low-grade lymphoma (e.g., follicular lymphoma)
  • CD20, CD30, CD79a, Ki-67
  • If Ki-67 > 90%, Richter transformation is occurring
A

Diffuse large B-cell lymphoma

305
Q
  • Low-grade B-cell (CD20) neoplasm, composed of small lymphocytes that efface the lymph node and are seen in circulation as “butt or soccer ball” cells
  • Incr lymphocytes and smudge cells
  • High N/C ratio and dense chromatin
  • CD5, CD20, CD23, CD43
  • May transform into diffuse large B-cell lymphoma (Richter transformation)
  • May develop autoimmune hemolytic anemia
  • MC leukemia overall
A

Chronic lymphocytic leukemia (CLL)

306
Q
  • Low-grade B-cell (CD20) lymphoma that expands the marginal zone
  • Assoc w/ chronic inflammatory states such as Hashimoto’s thyroiditis, Sjogren’s, and H. pylori infection
  • Monocytoid B-cells
  • Monotypic B-cells express IgM
  • slg
A

Marginal zone lymphoma (MZL)

307
Q
  • Small B-cell (CD20) lymphoma that forms follicle-like nodules
  • Presents in late adulthood w/ painless LAD
  • Driven by t(14;18)
  • CD10, CD20, CD22, BCL2, BCL6
  • May transform into DLBC lymphoma
A

Follicular lymphoma

308
Q
  • Aggressive B-cell (CD20) lymphoma that expands the mantle zone
  • Presents in late adulthood with painless LAD
  • Driven by t(11;14)
  • Overexpression of Cyclin D1 facilitates neoplastic proliferation
  • Monomorphic cells efface lymph node
  • Commonly involves GI tract and BM
  • CD5+, CD23–, CD43+, Cyclin D1 (+)
A

Mantle cell lymphoma

309
Q
  • Small B-cell (CD20) neoplasm, usually involves BM, lymph nodes, and spleen
  • Dutcher bodies and lots of plasma cells
  • Expresses B-cell Ag’s and Ig’s
  • Assoc w/ Hep C and cryoglobulinemia ***
  • Waldenstrom’s macroglobulinemia (IgM monoclonal gammopathy) seen in many cases
A

Lymphoplasmacytic lymphoma (LPL)

310
Q
  • Malignant proliferation of plasma cells in BM
  • Lytic (punched-out) lesions seen on x-ray, especially in vertebrae and skull
  • Elevated serum protein due to plasma cells producing Ig; M spike present on serum protein electrophoresis (SPEP)
  • Rouleaux formation
  • Primary AL amyloidosis
  • Proteinuria – free light chain excreted in urine as Bence-Jones protein
A

Multiple myeloma

311
Q
  • Plasma cell neoplasm
  • Solitary lesion in the BM cavity or in soft tissue
  • M-protein elevation
A

Solitary myeloma

“Plasmacytoma”

312
Q
  • MC type of myeloma
  • Incr serum protein w/ M spike on SPEP
  • M protein < 3 g/dL
  • Common in elderly
  • Patients are asymptomatic, but will eventually progress to multiple myeloma
A

Monoclonal gammopathy of undetermined significance

MGUS

313
Q
  • A single clone of plasma cells is responsible for overproduction of kappa chains or, rarely, lambda chains
  • Bence-Jones protein (light chain) found in the urine
A

Light chain dz

314
Q
  • Disorder in which Ig’s precipitate out into the blood at low temps
  • They DO NOT agglutinate RBCs
  • Assoc w/ plasma cell dyscrasia, macroglobulinemia, hep C, SLE, RA
A

Cryoglobulinemia

315
Q

What are the 4 Hodgkin lymphoma subtype CD markers?

A

CD15+, CD30+, EMA+, CD45–

316
Q

In what plasma cell dz do we see M protein > 3 g/dL?

A

Smoldering myeloma

317
Q
  • Neoplastic proliferation of Reed-Sternberg (RS) cells, which are large B-cells with multilobed nuclei and prominent nucleoli (“owl-eyed nuclei”)
  • Classically CD15+ and CD30+
  • Often assoc w/ eosinophilia
  • Reactive inflammatory cells (T, B, and dendritic cells) make up the bulk of the tumor and form the basis for classification of subtypes
A

Hodgkin lymphoma (HL)

318
Q

What are the 4 Hodgkin lymphoma subtypes?

A

Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Lymphocyte-depleted

319
Q
  • MC of all Hodgkin lymphoma subtypes
  • Classic presentation: enlarging cervical or mediastinal lymph node in a young adult, usually female
  • Lymph node is divided into bands of sclerosis
  • RS cells present in lake-like spaces (lacunar cells)
  • Eosinophilia
  • CD15+, CD30+, EMA+, CD45–
A

Nodular sclerosis HL

320
Q
  • Best prognosis of all HL subtypes
  • Frequent mononuclear cells and RS cells
  • Background of T-lymphocytes
  • CD15+, CD30+, EMA+, CD45–, 40% EBV (+)
A

Lymphocyte-rich HL

321
Q
  • HL subtype that is assoc w/ abundant eosinophils (RS cells produce IL-5)
  • CD15+, CD30+, EMA+, CD45–, 70% EBV (+)
A

Mixed cellularity HL

322
Q
  • Most aggressive of all HL subtypes
  • Usually seen in elderly
  • Assoc with HIV/AIDS
  • -CD15+, CD30+, EMA+, CD45–, and most are EBV (+)
A

Lymphocyte-depleted HL

323
Q
  • Usually a large single tumor nodule
  • Neoplastic L&H cells (CD15–, CD30–, CD20+, LCA+, Oct2+, Bob1+, EBV (–)
  • CD3+ cells form rosettes around the neoplastic L&H cells (“popcorn cells”)
  • Not part of the classical Hodgkin disease
  • Young males with cervical or axillary LAD, mediastinal dz
A

Nodular lymphocyte predominant HL

324
Q
  • Classic example = DiGeorge syndrome
  • Assoc w/ 22q11.2, giving rise to malformations of the thymus, parathyroids (hypoplasia), hypocalcemia, and diminished T-cell immunity
  • Also seen in SCID
A

Thymic hypoplasia

325
Q
  • Seen in myasthenia gravis and other autoimmune dz’s

- May be mistaken for thymoma and unneeded surgery may be performed

A

Thymic hyperplasia

326
Q
  • A product of epithelial cells

- Usually composed of the medullary type epithelial cell (spindled or elongated in shape)

A

Non-invasive thymomas

327
Q
  • Benign neoplasm that’s locally invasive but cytologically benign
  • Invasion occurs through the capsule into local structures surrounding the thymus
A

Invasive thymomas

328
Q
  • True carcinoma of the thymus
  • May be squamous cell carcinoma
  • May be a lymphoepithelial-like carcinoma
A

Thymic carcinoma

329
Q

CD11c:

A

Granulocyte, monocyte, and macrophages

Also expressed on hairy cell leukemias

330
Q

CD13:

A

Immature and mature monocytes and granulocytes

331
Q

CD14

A

Monocytes

332
Q

CD15:

A

Granulocytes and Reed-Sternberg (RS) cells

333
Q

CD33:

A

Myeloid progenitors and monocytes

334
Q

CD64:

A

Mature myeloid cells

335
Q

CD16:

A

NK cells and granulocytes

336
Q

CD56:

A

NK cells and a subset of T-cells

337
Q
  • Neoplastic accumulation of immature myeloid cells (blasts)
  • BM failure, anemia, thrombocytopenia/neutropenia
  • Increased blasts on PS > 20%
  • TdT+
  • CD34, CD38, HLS-DR
A

Acute myeloid leukemia (AML) with minimal differentiation (M0)

338
Q
  • Neoplastic accumulation of immature myeloid cells (blasts)
  • BM shows > 90% blasts
  • May see some granules or rare Auer rods
  • MPO+, SSB+ > 3%
  • CD13, CD33, CD34, CD117, HLA-DR
A

AML without maturation (M1)

339
Q
  • Neoplastic accumulation of immature myeloid cells (blasts)
  • BM and PS show maturation of cells (> 20% blasts, > 10% maturing neutrophils, 3%
  • CD11b, CD13, CD15, CD23, CD33, CD65, HLA-DR
A

AML with maturation (M2)

340
Q
  • Neoplastic accumulation of immature myeloid cells (blasts)
  • Characterized by t(15:17), which disrupts retinoic acid receptor (RAR) … RAR blocks maturation and promyelocytes (blasts) accumulate
  • Kidney bean-shaped nucleus
  • Short, thick Auer rods with stubby ends
  • Increased # of azurophilic granules
  • Strong assoc w/ DIC!
  • Tx: Vitamin A derivative
A

Acute promyelocytic leukemia (APL)

341
Q
  • Myeloid and lymphoid neoplasm with eosinophilia
  • Abnormalities of PDGFA, PDGFRB, or FGFR1
  • Rare dz
  • Share a fusion of a tyrosine kinase gene ***
  • Characteristic eosinophilia
  • Deletion of chr. 4 FLIP1L1 - PDGFRA
A

AML eosinophilia

342
Q
  • Rare dz
  • Assoc w/ hyperhistaminemia due to increased basophils
  • Causes: allergic, inflammatory disorders, endocrinopathy, renal dz, infections, irradiation, carcinoma
  • CD11b, CD13, CD33, CD123, CD203
A

Acute basophilic leukemia

343
Q
  • A proliferation of both neutrophil and monocyte precursors, including promonocytes (> 20%)
  • MPO+, NSE+
  • CD13, CD15, CD33, CD65
  • CD4, CD11b, CD11c, CD14, CD36, CD64, CD163, lysozyme
A

Acute myelomonocytic leukemia

344
Q
  • Neoplastic accumulation of monoblasts (> 80%)
  • Blasts characteristically infiltrate gum&raquo_space; Gingival hyperplasia ***
  • Hemophagocytosis may be seen
  • CD13, CD33, CD15
  • CD4, CD11b, CD11c, CD14, CD36, CD64, CD163, lysozyme
A

Acute monoblastic and monocytic leukemia

345
Q
  • Proliferation of megakaryocytes
  • SBB–, CAE–, MPO–
  • May react with PAS, acid phosphatase and focal or punctate non-specific esterase
  • CD41 and CD61 ***
A

Acute megakaryocytic leukemia

346
Q
  • Another name for myeloid sarcoma or extramedullary myeloid tumor
  • Very rarely can these occur without pre-existing conditions
  • Usually assoc w/ other AML
  • Leukemia cutis = infiltration of the dermis by leukemic cells, granulocytic sarcoma
A

Chloroma

347
Q

3 disorders seen:

  • Transient myeloproliferative disorder with large numbers of circulating blasts; GATA1 mutation
  • A true megakaryocytic leukemia; GATA1 mutation; ALL or AML can develop
  • An interval happens, then…..
A

Downs-related myeloid disease

348
Q
  • Neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors
  • Basophilia
  • Driven by t(9;22)(q34;q11.2), which generates a BCR-ABL fusion protein with increased tyrosine kinase activity *
  • No dysplasia is seen
  • Splenomegaly is common
A

Chronic myeloid leukemia (CML)

349
Q

How do you know when CML has begun the transformed and progressive phases?

A

Blast count > 20% of the PS or BM.

350
Q
  • Neoplastic proliferation of mature myeloid cells, especially RBCs
  • Males: Hb > 18.5 …. Females: Hb > 16.5
  • Assoc w/ JAK2 V617F mutation
  • Hypercellular BM
  • Low EPO
  • PS shows leukoerythroblastosis
A

Polycythemia vera

351
Q
  • Neoplastic proliferation of mature myeloid cells, especially megakaryocytes
  • Assoc w/ JAK2 kinase mutation (50% of cases)
  • Megakaryocytes produce excess platelet-derived growth factor (PDGF)&raquo_space; marrow fibrosis
  • Hepatosplenomegaly
  • Tear-drop RBCS
  • Increased LDH and anemia
A

Primary myelofibrosis

352
Q
  • Neoplastic proliferation of mature myeloid cells, especially platelets
  • Symptoms related to an increased risk of bleeding and/or thrombosis (thrombocytosis > 450X)
  • Packed marrow with megakaryocytes
A

Essential thrombocythemia

353
Q
  • Rare dz, derived from histiocytic or dendritic cells
  • Histiocytic sarcoma
  • Tumors arising from LAngerhans cells
  • Interdigitating dendritic cell sarcoma
  • Disseminated juvenile xanthogranuloma
A

Mast / dendritic cell dz

354
Q
  • A clonal hematopoietic stem cell dz characterized by cytopenias and dysplasia in one or more major myeloid cell lines
  • Ineffective hematopoiesis
  • Increased risk for developing leukemia
  • Key feature = dysplasia ***
  • Increased apoptosis
  • Ringed sideroblasts seen in some forms
  • Usually older pts
A

Myelodysplastic syndrome

355
Q
  • T-cell lymphoma that strongly and uniformly expresses CD30 ***
  • 2 major types based on expression of ALK protein
  • Represents about 2% of adult non-Hodgkin lymphomas and 20-30% in children
  • Can be nodal or extranodal
A

Anaplastic large cell lymphoma

356
Q
  • Neoplastic accumulation of precursor B-cells (B lymphoblasts) in BM
  • MC type of ALL
  • Characterized by lymphoblasts (TdT+)
  • Always involves BM
  • Lymphoblasts = PAS+, MPO– (not myeloid)
  • CD10, CD19, CD79a, TdT+
A

B-cell acute lymphocytic leukemia

B-ALL

357
Q
  • Neoplastic accumulation of precursor T-cells (T lymphoblasts) in BM
  • Always involves BM
  • Lymphoblasts = PAS+, MPO
  • CD1a, CD3c, CD7
A

T-cell acute lymphocytic leukemia

T-ALL

358
Q
  • Neoplasm composed of mature B-cells characterized by hairy cytoplasmic processes
  • (+) for tartrate-resistant acid phosphatase stain (TRAP+) ***
  • Hepatosplenomegaly *
  • “Dry tap” on BM aspiration *
  • BRAF
  • CD11c, CD20, CD25, CD103, CD123
A

Hairy cell leukemia

359
Q
  • Neoplastic proliferation of mature CD4+ T-cells that infiltrate the skin, producing localized skin rash, plaques, and nodules
  • Aggregates of neoplastic cells in the epidermis are called Pautrier microabscesses
  • Cells can spread to involve the blood, producing Sezary syndrome
  • CD2, CD3, CD4, CD5, CD8, TCRß
  • T-cell genes are rearranged *
A

Mycosis fungoides

360
Q

Triad:

  • Erythroderma
  • Generalized LAD
  • Circulating clonal T-cells with cerebriform nuclei ***
  • Thought of as end-stage mycosis fungoides
A

Sezary syndrome

361
Q

Primary effusion lymphoma. Think…

A

Kaposi’s