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Path Exam 3 Shuffled > Hematology > Flashcards

Hematology Flashcards

1
Q

describe the effect of 4 α genes deleted

A
  • 4 genes deleted = lethal in utero (hydrops fetalis)
    • γ chains form tetramers (Hb Barts) → extremely high affinity for O2 → no O2 reaches the tissues
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2
Q

the best screening test for the condition seen in the image is ____

A

the best screening test for the condition seen in the image is metabisulfite screen which causes cells with HbS to sickle

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3
Q

list the blood levels values seen in B12 deficiency

A
  • increased homocysteine
  • increased methylmalonic acid
  • low B12 (with normal folate)
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4
Q

___ is the most accurate test for the condition seen in the image, but the downside is ____

A

G6PD assay is the most accurate test for the condition seen in the image, but the downside is may have to wait 2 months b/c reticulocytes produced at time of crisis do NOT reflect the enzyme deficiency – only mature RBcs do

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5
Q

___ is the most common form of immunohemolytic anemia, with most antibodies being ____ class

A

warm antibody IHA is the most common form of immunohemolytic anemia, with most antibodies being IgG class

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6
Q

describe the etiology of idiopathic form of the condition seen in the image

A
  • idiopathic = 65% of cases
    • immune-mediated (cellular or T cell) destruction of antigenically altered stem cells
    • primary intrinsic stem cell defect
      • genetic abnormalities detected in some cases
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7
Q

describe lab findings seen in the condition in the image

A
  • blood
    • ↑ LDH
    • ↑ unconjugated bilirubin
    • ↑ free Hb
    • ↑ reticulocytes
    • ↓ haptoglobin (made by liver to bind free Hb)
  • urine
    • free hemoglobin → hemoglobinuria & hemosiderin → hemosiderinuria → dark urine
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8
Q

name causes of malabsorption that can lead to folate deficiency

A
  • malabsorption in the jejunum that can be caused by:
    • Crohns
    • Tropical sprue
    • Celiac disease
    • Whipples
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9
Q

describe the treatment for the condition seen in the image

A

splenectomy & folic acid supplements

will see HJ bodies upon splenectomy

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10
Q

describe how malabsorption can lead to B12 deficiency

A
  • malabsorption in ileum
    • absorption in distal ileum affected by:
      • Crohns
      • Tropical sprue
      • Celiac
      • Whipples
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11
Q

chronic ___ can lead to B12 deficiency

explain this concept

A

chronic pancreatitis can lead to B12 deficiency

  • pancreatic enzymes are needed to cleave R binder from B12 so intrinsic factor can bind to it
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12
Q

describe the osmotic fragility test in relation to the condition seen in the image

A
  • osmotic fragility test:
    • in 65% of patients with HS, RBCs lyse prematurely compared to normal when exposed to increasingly hypotonic solution
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13
Q

describe the presentation of the condition seen in the image

A
  • asymptomatic until exposed to injurious agent
  • 2-3 days after oxidant stressor: acute hemolysis, fever, jaundice, fatigue, dark urine (due to free Hb)
  • NO splenomegaly (differential from HS due to being intrinsic)
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14
Q

describe the complications of the condition seen in the image

A
  • complications:
    • CHF
    • if they have Plummer Vinson → sq. cell carcinoma of esophagus
    • Pica syndrome (psychiatric problem w/ eating dirt, ice)
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15
Q

describe the etiology of the condition seen in the image

A
  • etiology = intrinsic/genetic defect in RBC membrane proteins
    • ankyrin (AD)
    • spectrin (AR)
    • band 3.1
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16
Q

describe the presentation of the condition seen in the image

A
  • mild to chronic hemolytic anemia, jaundice, fatigue, dragging sensation in LUQ due to splenomegaly
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17
Q

in PNH there is absence of CD__, CD___ and ____ which causes susceptibility to ____

A

in PNH there is absence of CD55, CD59 and C8-binding protein which causes susceptibility to hemolysis

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18
Q

cardiac ____ can cause hemolysis due to mechanical RBC damage

A

cardiac prosthetic valves can cause hemolysis due to mechanical RBC damage

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19
Q

describe causes of anemia in chronic diseases (3 main groups)

A
  1. chronic bacterial infections
    • lung abscess, endocarditis
  2. chronic immune disorders
    • RA
  3. malignant tumors
    • cancers of lung & breast, lymphoma
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20
Q

describe chronic primary immune thrombocytopenia (ITP)

A
  • chronic:
    • women 20-40
    • insidious onset of skin +/- mucosal bleeding
    • rarely resolves spontaneously
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21
Q

describe the type of anemia seen in the image

A

normocytic anemia with predominant extravascular hemolysis

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22
Q

describe the pathogenesis of PNH

A
  • PIGA forms an enzyme needed to synthesize GPI
  • 3 GPI-linked proteins normally inhibit complement activation on blood cells; absence of CD55, CD59 and C8-binding protein causes susceptibility to hemolysis
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23
Q

in diffuse liver disease, there is ___ hypofunction, affecting mainly ____, and the etiology is multifactorial

A

in diffuse liver disease, there is BM hypofunction, affecting mainly RBCs, and the etiology is multifactorial

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24
Q

list causes of microangiopathic hemolytic anemia (5)

A

DIC, malignant HTN, SLE, TTP/HUS, disseminated cancer

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25
Q

list 2 potential treatments for chronic ITP

A
  • immunosuppression via steroids
  • splenectomy
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26
Q

describe a diet that is at greater risk of acquiring the condition seen in the image

A
  • nutritional: iron from plants (non-heme) & animal (heme = better absorbed)
    • vegan more likely to get IDA than B12 def.
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27
Q

describe the complications of the condition seen in the image

A
  • all anemias → LVH → LHF → RHF → CHF
  • rapid production of RBCs uses folate → folate def. anemia → megaloblastic anemia
  • increased risk of aplastic crisis due to Parvovirus B19 (infects normoblasts/erythroid precursors) → reticulocytopenia in chronic hemolytic anemia
  • bilirubin gallstones → cholecystitis due to supersaturation of bilirubin in bile → increased chance of precipitation
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28
Q

____ is the leading cause of death in PNH (40% develop ____)

A

thrombosis is the leading cause of death in PNH (40% develop DVT)

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29
Q

the ____ response can be used to determine B12 deficiency

explain this

A

the reticulocyte response can be used to determine B12 deficiency

there would be improvement approx. 5 days after a parenteral B12 injection

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30
Q

in the condition seen in the image, ___ is necessary to rule out other causes of pancytopenia

A
  • BM biopsy is necessary to rule out other causes of pancytopenia (acute leukemia, myelodysplastic syndrome)
  • BM biopsy
    • very hypocellular
      • only fat cells, few lymphocytes, plasma cells
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31
Q

describe the lab values seen in ACD and IDA

A
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32
Q

50% of patients with warm antibody IHA have predisposing factors, such as ___, ___ or ____

A

50% of patients with warm antibody IHA have predisposing factors, such as autoimmune diseases, lymphoma or drug reactions

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33
Q

a small number of PNH patients develop ____ or _____

A

a small number of PNH patients develop acute myeloid leukemia or myelodysplastic syndrome

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34
Q

describe the acquired causes of the condition seen in the image (3)

A
  • chemical agents:
    • dose-related, predictable
      • alkylating agents and antimetabolites (chemotherapeutic agents)
    • idiosyncratic
      • chloramphenicol, chlorpromazine, phenytoin
  • physical agents:
    • whole body irradiation
  • viral agents:
    • hepatitis D and E, CMV, EBV, herpes zoster
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35
Q

describe acute primary immune thrombocytopenia (ITP)

A
  • acute:
    • children
    • post-viral
    • abrupt onset
    • spontaneous resolution
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36
Q

describe the pathogenesis of the condition seen in the image

A
  • ↓ iron → ↓ heme → ↓ Hb → increased cell division → microcytic hypochromic anemia
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37
Q

describe the etiology of the major form of the condition seen in the image

A
  • point mutations of chr. 11
    • absent B chain (B° caused by mutation in splicing/chain termination)
    • diminished B chains (B+ caused by mutation in the promoter region)
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38
Q

describe the image

A

G6PD

as the splenic macrophages pluck out the Heinz bodies inclusions, “bite cells” are produced

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39
Q

describe the image

A

G6PD

red cells with precipitates of denatured globin (Heinz bodies) shown by supravital staining

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40
Q

list a treatment for hemophilia A and a potential complication

A
  • recombinant FVIII infusions
    • 15% develop FVIII antibodies
    • also risk of transmission of viral diseases
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41
Q

describe the presentation of the condition seen in the image in adults

A

sickle cell anemia

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42
Q

___ a peptide synthesized in the ___ in response to high levels of iron stores, inhibits iron uptake from ___ mucosal cells into the bloodstream

A

hepcidin a peptide synthesized in the liver in response to high levels of iron stores, inhibits iron uptake from duodenal mucosal cells into the bloodstream

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43
Q

list 2 possible treatments of PNH

A
  • immunosuppression
  • bone marrow transplantation
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44
Q

describe the type of anemia seen in the condition in the image

A

normocytic anemia with predominant intravascular hemolysis (due to abnormality in HMP shunt)

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45
Q

describe the type of anemia that is seen in the image

A

normocytic anemia with predominant extravascular hemolysis

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46
Q

if the flow cytometry image shown is normal, what is seen in PNH?

A
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47
Q

describe the complications of the condition seen in the image

A
  • aplastic crisis → due to Parvovirus B19
  • folic acid deficiency due to chronic hemolytic anemia
  • bacterial infections from iron-loving bacteria (Vibrio & Yersinia enterocolytica)
  • bilirubin gallstones
  • failure to thrive
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48
Q

list causes of decreased production of thrombocytes (thrombocytopenia(

A
  • generalized BM disease
  • aplastic anemia
  • marrow infiltration
    • leukemia, metastatic cancer, granulomatous inflam.
  • drug-induced
    • ethanol, cytotoxic drugs
  • infections
    • HIV, measles
  • megaloblastic anemia
  • myelodysplastic syndromes (MDS)
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49
Q

describe blood level values seen in folate deficiency

which value is truly diagnostic of folate def.?

A
  • increased homocysteine
    • increased in both, folate and B12 def.
  • decreased serum folate
    • only truly diagnostic test, as other changes could mean B12 def. too
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50
Q

describe how nutrition can lead to folate deficiency

A

lack of raw greens (boiling destroys folate)

most common cause of folate deficiency in older patients

51
Q

describe conditions of chronic blood loss that can lead to the condition seen in the image

A
  • chronic blood loss:
    • right-sided colon cancer → occult bleeding
    • peptic ulcer by H. pylori → melena
    • uterine fibroids → heavy bleeding (menorrhagia)
52
Q

list the most common outcome and complications of the condition seen in the image

A

self-limiting: complete resolution

  • usually self-limiting with complete resolution after hemolytic event
  • Hb (as is hemosiderin) is toxic to renal tubules → acute tubular necrosis → acute renal failure
53
Q

describe how the Schilling test can be used to determine B12 malabsorption

A
  • Schilling test = inability to absorb an oral dose of B12, measured by urinary excretion of labeled B12
54
Q

describe the type of anemia seen in the image and why it is significant

A

microcytic hypochromic anemia

most common cause of anemia

55
Q

list the 5 features found in thrombotic thrombocytopenic purpura (TTP)

A
  • clinical syndrome of:
    • fever
    • thrombocytopenia
    • microangiopathic hemolytic anemia
    • transient neurologic deficits
    • renal failure
56
Q

describe the most accurate test for diagnosing the condition seen in the image

A
  • most accurate test = BM biopsy w/ Prussian blue (depletion of BM iron stores) → mild erythroid hyperplasia
57
Q

how is HUS different from TTP?

A

clinically, HUS is like TTP, except HUS has NO neurologic deficits and instead has prominent acute renal failure

58
Q

describe the pathogenesis of the condition seen in the image

A
  • pathogenesis: HbS polymerizes when deoxygenated → polymers aggregate into needle-like structures → sickle cells
59
Q

describe the etiology of the condition seen in the image and name the 2 types of mutations

A
  • etiology = X-linked recessive
  • 2 types of mutations (role against falciparum malaria)
    • G6PD A (African variant) = mild version → only older RBCs are affected
    • G6PD (Mediterranean variant) = more severe → even younger RBCs are affected
60
Q

describe how pernicious anemia can lead to B12 deficiency

A
  • pernicious anemia = antibodies destroying intrinsic factor = type II HS
    • most common cause in the US
    • pernicious anemia → intestinal gastric adenocarcinoma & type I gastric carcinoid
61
Q

name drugs that can lead to folate deficiency

A

phenytoin, methotrexate (chemotherapy)

62
Q

describe diseases that cause malabsorption that can lead to the condition seen in the image

A
  • malabsorption: absorption in duodenum affected by Crohns, tropical sprue, celiac disease and Whipple
    • iron balance regulated at the level of absorption by hepcidin (inhibits iron uptake from duodenal mucosal cells into the bloodstream)
63
Q

describe the blood investigations found in the major form of the condition seen in the image

A
  • blood:
    • ↑ LDH
    • ↑ reticulocytes
    • ↑ RDW
    • ↑ serum iron & ferritin
    • ↓ TIBC
64
Q

describe the pathogenesis and presentation of the major form of the condition seen in the image

A
  • no B chains (no HbA which is α2B2), which leads to an increase in HbF (α2γ2) and HbA2 (α2δ2)
  • expansion of hematopoietic marrow in skull → chipmunk face, frontal bossing & crew-cut appearance due to erythroid hyperplasia in flat bones
  • intramedullary hemolysis → ineffective erythropoeisis b/c α chains are not paired with B chains → α chains precipitate and form tetramers
  • extravascular hemolysis: splenomegaly, fatigue, jaundice, brown discoloration of skin due to iron overload
65
Q

list the 2 acquired platelet function abnormalities

A
  • drugs:
    • aspirin and NSAIDs
  • uremia
    • chronic renal failure
66
Q

describe genetic composition in B-thalassemia minor vs. B thalassemia major

A
  • B-thalassemia minor = (B/B+) = mild asymptomatic anemia
  • B-thalassemia major = (B°/B°), (B+/B+), (B+, B°) = severe transfusion-dependenent anemia; abnormal Hb causes occlusions in small blood vessels
67
Q

name conditions where there is an increased demand for folate

A

increased demand during pregnancy, cancer, and chronic hemolytic anemia

68
Q

describe type I and type III vs. type II of von Willebrand disease

A

I and III = reduced quantity of vWF

II = qualitative (functional) abnormality of vWF

69
Q

PNH is caused by a mutation in ____

A

PNH is caused by a mutation in PIGA (X-linked)

70
Q

describe what is seen on peripheral blood smear of the major form of the condition seen in the image

A
  • PB:
    • target cells, normoblasts, reticulocytes, anisocytosis, poikilocytosis due to extramedullary hepatopoeisis
71
Q

describe the etiology of the condition seen in the image

A
  • AR point mutation at position 6 of B-globin
    • glutamic acid (polar) replaced by valine (neutral/non-polar) → removes biconcavity
  • homozygotes (HbS, HbS) = sickle cell anemia
72
Q

for the condition seen in the image,

younger patients can be treated with ____

while

older patients can be treated with ____

A

younger patients can be treated with bone marrow transplant

while

older patients can be treated with immunosuppression

73
Q

most iron is absorbed in the ____

a small fraction enters the blood, but most remains in ___ and is lost by ____

A

most iron is absorbed in the duodenum

a small fraction enters the blood, but most remains in intestinal cells and is lost by intestinal exfoliation

74
Q

in the condition seen in the image, the most common predisposing factor for episodes is ____

A

in the condition seen in the image, the most common predisposing factor for episodes are infections

NADPH is needed for resp. burst

75
Q

describe the presentation of the condition seen in the image in children

A
  • children:
    • crew-cut appearance & chipmunk face (due to hematopoiesis in skull)
    • failure to thrive
    • dactylitis (swollen hands and feet due to vaso-occlusive infarcts in bones → aseptic necrosis of metacarpal bones)
    • spleen (crisis → splenomegaly → autosplenectomy due to fibrosis → recurrent encapsulated bacterial infxn = most common cause of death in children)
76
Q

describe 1 α-gene deletion

A

gene deletions on chr. 16

  • 1 gene deleted = asymptomatic
77
Q

describe the cause of sickling and types of crises in the condition seen in the image

A
  • crisis (increased sickling) caused by: hypoxia, acidosis, infection, dehydration, hypothermia, HbC (glutamic acid is replaced by lysine)
  • types of crises: painful vaso-occlusive, hemolytic, splenic sequestration & aplastic crisis
78
Q

describe the treatment for the condition seen in the image

A
  • bone marrow transplant
  • hydroxyurea (stimulates HbF, increased NO, decreased platelet aggregation)
  • vaccines after splenectomy
  • folic acid
79
Q

describe the lab findings in the condition seen in the image

A
  • ↑ reticulocytes (reticulocytosis)
  • ↑ LDH
  • ↑ MCHC (only condition that has this)
  • ↑ RDW
  • ↑ unconjugated bilirubin
  • ↓ MCH
80
Q

a PB smear in chronic ITP may show large ____ which indicates ____

A

a PB smear in chronic ITP may show large platelets which indicates rapid BM turnover

81
Q

the test used for definitive diagnosis of the condition seen in the image is ____

A

the test used for definitive diagnosis of the condition seen in the image is hemoglobin electrophoresis (most accurate)

82
Q

describe the change in SA:V ratio in the condition seen in the image and what it affects

A
  • decreased SA:V ratio (spheres) → pack more Hb → no central pallor → increased MCHC (due to cell becoming more concentrated) & increase in RDW
83
Q

in myelophthisic anemia, there is BM replacement by ____ that disturb ___ (often with ____)

A

in myelophthisic anemia, there is BM replacement by abnormal infiltrates that disturb normal architecture (often with fibrosis)

84
Q

in the condition seen in the image, ___ can deliver O2 normally but ___ recognizes them as foreign

A

in the condition seen in the image, spherocytes can deliver O2 normally but the spleen recognizes them as foreign → chronic extravascular hemolysis

85
Q

cold antibody hemolytic anemia is mainly caused by ___ antibodies at ___ temperatures

A

cold antibody hemolytic anemia is mainly caused by IgM antibodies at cold temperatures

IgM = pentamer, so able to interact more and don’t need heating up to cause agglutination

86
Q

there is a common association between PNH and ____

and ___ can also be a problem

A

there is a common association between PNH and aplastic anemia

and iron deficiency can also be a problem

87
Q

describe the pathogenesis of the condition seen in the image

A
  • pathogenesis:
    • reduced RBC membrane stability → loss of small fragments during normal shearing stresses in the blood circulation → spherocytosis to accommodate → unable to traverse the splenic sinusoids → phagocytosis and destruction by splenic macrophages → splenomegaly usually seen due to functional hypertrophy
88
Q

describe what is seen in blood serum levels in the condition seen in the image

A
  • blood:
    • ↑ reticulocytes
    • ↑ LDH
    • ↑ unconjugated bilirubin
    • ↑ free Hb (dark urine)
    • ↓ haptoglobin
89
Q

describe the diagnosis of the major form of the condition seen in the image

A
  • diagnostic: Hb electrophoresis → no A band b/c no B chain, prominent F and normal/slightly elevated A2
90
Q

list clinical features of PNH

A
  • clinical features:
    • intravascular hemolysis: chronic low-grade hemolysis
    • thrombosis: platelet dysfunction → prothrombotic state
91
Q

describe the presentation of B12 deficiency

A
  • presentation:
    • fatigue
    • atrophic glossitis
    • cheliosis
    • neurological symptoms
      • decreased vibration & proprioception due to subacute combined degeneration of dorsal column due to methylmalonic acid
92
Q

list the stages of the condition seen in the image

A
  • stages:
    • storage iron depleted: ↓ ferritin; increased TIBC
    • serum iron depleted: ↓ serum iron; ↓ % transferrin sat.
    • normocytic anemia: bone marrow makes FEWER, NORMAL RBCs
    • microcytic, hypochromic anemia: bone marrow makes smaller and fewer RBCs
93
Q

describe what is seen on PB smear of the condition seen in the image

A
  • PB: anisopoikilocytosis (pencil cells), hypochromic microcytic cells and ↑ platelets
94
Q

describe the type of anemia seen in the condition in the image

A

hypochromic microcytic anemia with extravascular hemolysis

95
Q

a BM biopsy in chronic ITP would show increased ____

A

a BM biopsy in chronic ITP would show increased megakaryocytes

96
Q

describe the peripheral blood smear in the condition seen in the image

A
  • PB:
    • pancytopenia
    • normochromic normocytic anemia
    • very low reticulocyte count
97
Q

describe laboratory findings in PNH

A
  • hemoglobinuria, hemosiderinuria
  • older lab tests:
    • sucrose hemolysis test (screening)
    • Ham’s acid hemolysis test
  • flow cytometry:
    • absence of CD55, CD59 on WBCs
    • Flaer test
98
Q

in chronic renal failure, there is decreased production of _____

A

in chronic renal failure, there is decreased production of erythropoietin

99
Q

describe the image

A

hereditary spherocytosis

note the anisocytosis and several hyperchromic spherocytes

Howell-Jolly bodies (small nuclear remnants) are also present in the red cells of this asplenic patient (seen on the right)

100
Q

list the 4 causes of iron deficiency

which is the most common in the Western world?

A
  1. dietary deficiency
  2. impaired absorption
  3. increased requirements
  4. chronic blood loss
    • commonest cause of iron def. in the Western world
      • where iron def. anemia is due to chronic GI blood loss until proven otherwise
101
Q

describe the effect of 2 α genes deleted

A
  • 2 genes deleted = miild anemia w/ increased RBC count
    • cis deletion = increased risk of severe thalassemia in offspring
102
Q

describe what is seen and what it is caused by

A

schistocytes are seen which can be caused by microangiopathic hemolysis

103
Q

why is thrombosis seen in PNH?

A

platelets are also missing GPI (and therefore CD55, CD59) and are susceptible to complement-mediated killing

the destroyed platelets release cytoplasmic contents into circulation, causing thrombosis

104
Q

list the 4 categories of thrombocytopenia

A
  1. decreased platelet production
  2. decreased platelet survival
  3. sequestration (splenic)
  4. dilutional
105
Q

describe type III von Willebrand disease

A
  • type III = severe deficiency/lack of vWF
    • affects Factor VIII stability int he plasma so the patient may clinically present like Hemophilia A (increased PTT)
106
Q

list the laboratory findings in chronic ITP

A
  • decreased platelets
  • increased bleeding time
  • autoantibodies against platelet membrane glycoproteins
107
Q

myelophthisic anemia can be caused by ____ & ____ and there is a ____ blood picture

A

myelophthisic anemia can be caused by metastatic cancer & granulomatous inflammation and there is a leukoerythroblastic blood picture

108
Q

in the condition seen in the image, there is an increased risk of aplastic crisis due to ____

A

in the condition seen in the image, there is an increased risk of aplastic crisis due to Parvovirus B19 which infects normoblasts/erythroid precursors

109
Q

describe what is seen on PB smear in folate deficiency

A
  • hypersegmented neutrophils (>5 lobes)
  • increased MCV
  • decreased reticulocytes
  • normal ferritin, TIBC
110
Q

describe blood level values seen in anemia of chronic disease

A
  • decreased serum iron
  • decreased TIBC
  • increased serum ferritin
  • normal or increased iron stores in BM
  • EPO levels inappropriately low
111
Q

describe the blood values in the condition seen in the image

A
  • blood:
    • ↓ iron & ferritin
    • ↑ TIBC
    • ↑ platelets
    • ↑ free erythrocyte protoporphyrin
112
Q

spontaneous remission of the condition seen in the image is uncommon in adults, but in children a ____ is more likely with a full recovery

A

spontaneous remission of the condition seen in the image is uncommon in adults, but in children a viral etiology is more likely with a full recovery

113
Q

the most common cause of death in the major form of the condition seen in the image is ____

A

the most common cause of death in the major form of the condition seen in the image is heart failure due to 2° hemochromatosis (due to increased blood transfusions)

114
Q

describe nutritional deficiencies that can lead to B12 deficiency

A
  • nutritional: B12 found in dairy products & meat, therefore vegans can develop B12 deficiency
115
Q

describe the presentation of the condition seen in the image

A
  • presentation:
    • asymptomatic
    • weakness
    • pallor
  • koilonychia (spooning of fingernails), ↓ reticulocytes due to no iron, ↓ MCV, ↓ MHC, ↓ MCHC, ↑ RDW
116
Q

describe how Plummer Vinson syndrome can lead to the condition seen in the image

A
  • PVS triad:
    • IDA
    • esophageal webs
    • atrophic glossitis
117
Q

describe type I and II von Willebrand disease

A
  • type I = 70% of all cases of vWD, with mild, mostly mucosal bleeding
  • type II = 25% of vWD with mild to moderate bleeding
118
Q

describe the pathogenesis of the condition seen in the image

A
  • pathogenesis: abnormal enzymes are misfolded
    • ↓ G6PDH → ↓ NADPH → ↓ GSH → oxidative injury by H2O2 → intravascular hemolysis
  • oxidative stressors = fava beans, infections/antibiotics, antimalarial & sulfa drugs
    • mechanism: oxidation of SH groups on globin chains of Hb → Hb precipitates as Heinz bodies → damage to RBC membrane → intravascular hemolysis (predominantly)
119
Q

___ is the most common cause of death in adults with the condition seen in the image

A

acute chest syndrome: necrotizing pneumonia due to vaso-occlusion of pulm. arteries is the most common cause of death in adults

120
Q

describe the lab findings in hemophilia A

A

factor VIII deficiency

  • normal platelets, BT, PT
  • prolonged PTT
121
Q

describe the impaired iron utilization and decreased RBC production seen in anemia of chronic disease

A
  • decreased transfer of iron from the BM storage pool (macrophages) to RBC precursors, which is caused by hepcidin
    • increased hepcidin production in chronic inflammatory states
  • decreased erythropoiesis with inappropriately low erythropoietin
122
Q

describe the complications of the condition seen in the image

A
  • prone to small vessel thrombosis → infarct
    • sickled cells are “sticky” due to increased expression of adhesion molecules on their surface
  • pooling of blood in the spleen aka sequestration crisis → rapid splenic enlargement and entrapment of sickled RBCs → hypovolemia → reticulocytosis present in peripheral blood → infarct → splenic fibrosis → autosplenectomy → HJ bodies
123
Q

describe the effect of 3 α genes deleted

A
  • 3 genes deleted = severe anemia
    • B chains form tetramers (HbH) → high affinity for O2 → severe tissue hypoxia
      • HbH is prone to oxidation → precipitated inclusions in older RBCs → extravascular hemolysis

Path Exam 3 Shuffled (9 decks)

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