Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Heme/Lymph II Exam II > Disorders of red blood cells, spleen, thymus (Puttoff) > Flashcards

Disorders of red blood cells, spleen, thymus (Puttoff) Flashcards

1
Q

what is in the red pulp of the spleen

A

Constitutional symptoms
fever, night sweats, or weight loss (tuberculosis, lymphoma, or other malignancy)
fever (majority of the infectious diseases)
Use of medications that can cause lymphadenopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is in the white pulp of spleen

A

where T cells and B cells are located – B cells found in the germinal center

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the 4 main functions of the spleen

A

• Phagocytosis of blood cells and particulate matter

• Antibody production
o Antibody secreting plasma cells are the in the sinuses of the red pulp mainly
o Includes antibodies against microbials, and autoantibodies

• Hematopoiesis
o During fetal development – minor site of hematopoiesis
o Compensatory extramedullary hematopoiesis in severe chronic anemia

• Sequestration of formed blood elements
o With splenomegaly→ more platelet mass is held producing thrombocytopenia.
o Enlarged spleen can also trap white blood cells and induce leukopenia

harbors 30-40% of platelet mass in the body (with splenomegaly up to 80-90% of platelets can be sequestered → thrombocytopenia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is nonspecific acute splenitis

A

Enlargement of the spleen in any blood-borne infection

Enlarged spleen and soft

Acute congestion of the red pulp- which can encroach on and efface the lymphoid follicles

Neuts, plasma cells, eosinophils are usually present

When the agent is strep, commonly the white pulp follicles may undergo necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what are the causes of congestive splenomegaly

A

–Chronic venous outflow obstruction → leads to splenic or portal vein HTN.

–Systemic or central venous congestion → seen in right heart failure

Cirrhosis of liver

Obstruction of extrahepatic portal vein or splenic vein → casued by spontaneous portal vein thrombosis (can be caused by infiltrating tumors from neighboring organs- stomach or pancreas)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what are the findings in congestive splenomegaly

what type of hemolysis might be found?

A

Marked enlargement of spleen

Thickened and fibrous capsule

Red pulp becomes fibrotic over time

Deposition of collagen in the basement membrane of sinusoids

Because there is slowing of blood flow from the cords to the sinusoids there is prolonged exposure of red blood cells to macrophages→ excessive destruction (hypersplenism)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the cause of splenic infarcts…

what do they look like

A

Usually caused by the occlusion of the major splenic artery

There is lack of collateral blood supply which predisposes the spleen to infarction following vascular occlusion

Bland infarcts→ pale, wedge-shaped, subscapular in location. Capsule is covered with fibrin

Septic infarcts→ suppurative necrosis. Large scars develop with healing

Infarcts are often from the heart**

Common in pt’s who have infectious endocarditis of the mitral or aortic valves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the 2 most common types of neoplasms in the spleen

A

lymphangiomas

hemangiomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
  • can be found anywhere in the abdominal cavity

- of great clinical importance in hereditary spherocytosis, and immune thrombocytopenia purpura

A

accessory spleen

congenital anomaly of spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what might cause splenic rupture

are chronically enlarged or acute enlarged spleens more likely to rupture

A

Usually precipitated by blunt trauma

Spontaneous rupture→ usually there is previously abnormal spleen → infectious mononucleosis, malaria, typhoid fever, lymphoid neoplasms:

  • these cause the spleen to enlarge rapidly producing a thin capsule that can rupture easily
  • perform splenectomy to prevent death from blood loss

Chronically enlarged spleens are less likely to rupture b/c of toughening/fibrosis of capsule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what types of cells are in the thymus

A

Thymic epithelial cells (form Hassall’s Corpuscles in medulla),

immature T lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

morphology of cortical peripheral epithelial cells

A

polygonal and have abundant cytoplasm with dendritic extensions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

morphology of medulla epithelial cells

A

Medulla epithelial cells are densely packed and have spindle-shaped, scant cytoplasm, no interconnecting processes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

thymic hypoplasia

A

DiGeorge syndrome- defects in cell mediated immunity and variable abnormalities of parathyroid development , 22q11 deletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

thymic cysts

  • what lines these cysts
  • what should you do if you find a cyst
A

discovered incidentally

  • rarely exceed 4 cm
  • lined by stratified to columnar epithelium

***presence of a cystic thymic lesion in a symptomatic pt should provoke a thorough search for a neoplasm (thymoma or lymphoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what occurs with thymic hyperplasia

what types of diseases/settings does this occur in

what are these often mistaken for

A

Appearance of B cell germinal centers within the thymus- thymic follicular hyperplasia

Occurs in:

  • chronic inflammatory states
  • Myasthenia gravis- 65-70% of cases
  • SLE
  • Graves
  • RA
  • Sclerodoma

May be mistaken radiologically for a thymoma leading to unnecessary surgical procedures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what cell type makes up thymomas

A

thymic epithelial cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what are the clinical features, etiology and location of thymomas

A

In all categories:

  • adults older than 40
  • rare in children
  • males = females
  • most arise in anterior superior mediastinum**
  • sometimes occur in neck, thyroid, pulmonary hilus, or elsewhere
  • uncommon in posterior mediastinum

40% present with symptoms stemming from impingement on mediastinal structures***

Another 35-40% are detected in the course of evaluating pt’s with myasthenia gravis ***

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

22q11 deletion

A

DiGeorge syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

tumors that usually have a substantial portion of medullary type epithelial cells are usually

A

noninvasive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

medullary type epithelial cells or mix of medullary type + cortical type

sparse infiltrates of thymocytes

makes up 50% of thymomas

A

tumors that are cytologically benign and non invasive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

thymoma
penetrates through the capsule into surrounding structures

epithelial cells are more likely to be cortical type –> abudant cytoplasm, rounded vesicular nuclei and mixed with numerous thymocytes

neoplastic cells may show atypia

20-25% of all thymomas

A

Tumors that are cytologically benign but invasive or metastatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what are the survival rates for tumors that are cytologically benign but invasive or metastatic

A

Extensive invasion → 5 year survival of less than 50%

With minimal invasion, complete excision yields 5 year survival of >90%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Tumors that are cytologically malignant (thymic carcinoma)

A

5% of thymomas

Fleshy, invasive masses, sometimes accompanied by metastases to sites like lungs

  • Most are squamous cell carcinomas
  • About 50% of these contain monoclonal EBV genomes

Atypia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what is anemia

A

Defined as a reduction of the total circulating red cell mass

Reduction in oxygen carrying capacity → tissue hypoxia

Usually diagnosed based on reduction in hematocrit and the hemoglobin concentration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

MCV >100

MCV <80

A

macrocytosis

microcytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what are the signs/symptoms of anemia

A

Pale
Weakness
Malaise
Easy fatigability

Dyspnea on mild exertion due to lowered O2 content

Hypoxia→ fatty changes in the liver, myocardium and kidney

Cardiac fatty changes can lead to cardiac failure and further hypoxia→ angina pectoris

Central nervous system hypoxia can cause headache, dimness of vision and faintness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

what occurs in acute blood loss?

what is seen in lab studies?

what type of anemia is this ?

A

Loss of fluid leads to shift in fluids from the interstitial fluid compartment → reduced hematocrit and hemodilution

Blood loss = decreased O2 content→ release of EPO from kidney → proliferation of committed erythroid progenitors (takes about 5 days for newly synthesized reticulocytes to appear in PB)

The effects of acute blood loss are mainly due to loss of intravascular volume, which if massive can lead to cardiovascular collapse, shock and death

Significant bleeding results in changes in blood involving red cells AND white cells and platelets.

Significant blood loss→ decrease in blood pressure→ adrenergic hormone release → mobilization of granulocytes → leukocytosis

Thrombocytosis

Usually starts as normocytic normochromic anemia → later on more reticulocytes appear (macrocytosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what is extravascular hemolysis

what are the clinical findings?

A

Caused by:
• Alterations that render the red cell less deformable
destruction of red cells in
phagocytes→

anemia,
splenomegaly,
jaundice,
variable decreases in haptoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

what is intravascular hemolysis

what are the clinical findings?

A

caused by:
•mechanical injury→ cardiac valves, thrombotic narrowing of microcirculation, bongo drum beating, marathon running

  • complement fixation- antibodies bind red cell antigens
  • intracellular parasites
  • exogenous toxic factors
Findings:
-anemia, 
hemoglobinemia, 
hemoglobinuria, 
hemosiderinuria, 
jaundice
-splenomegaly is not seen***
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

why does renal hemosiderosis occur

A

accumulation of iron released from hemoglobin accumulating in renal tubular cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

why does urine turn brown with hemolytic anemias?

A

As serum haptoglobin is used up to sequester free hemoglobin, the remaining free hemoglobin oxidizes to methemoglobin = brown in color→ can make urine turn red brown

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what are the lab findings in hemolytic anemias?

A
  • increased numbers of erythroid precursors (normoblasts) in marrow
  • reticulocytosis in PB
  • hemosiderin (iron containing pigment) in the spleen, liver, BM = hemosiderosis
  • Extramedullary hematopoiesis if anemia severe (in liver, spleen, lymph nodes)
  • Pigment gallstones–> due to excessive bilirubin excreted by the liver into the GI tract and converted to urobilin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

what are the 3 shared features in all hemolytic anemias

A
  • Short red cell life span
  • Elevated EPO
  • Accumulation of hemoglobin degradation products

pt’s often benefit from splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Caused by intrinsic defects in red cell membrane skeleton that render red cells spheroid, less deformable, and vulnerable to splenic sequestration and destruction

Autosomal dominant (75%) 
Remaining cases are compound heterozygosity (inheritance of 2 defects)

Can turn into an aplastic crisis

mutations:
ankyrin
band 3
spectrin
band 4.2

results in failure to produce any protein–> destabilizes the plasma membrane

Morphology:
dark staining red cells lacking central zone of pallor
-reticulocytes 
-marrow hyperplasia
-mild jaundice

Clinical features:
Northern europe descent
moderate splenomegaly ***
extravascular hemolysis

***increased MCHC

Anemia, splenomegaly, jaundice triad ***

A

Hereditary spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

what is an aplastic crisis

A

triggered by parvovirus

Parvovirus → infects and kills red cell progenitors causing red cell production to cease. Because of the reduced life span of HS red cells, cessation of erythropoiesis for even short time periods leads to sudden worsening of the anemia. Transfusions may be necessary to support the patient until the immune response clears the infection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Recessive X-linked trait (M>F)

results in decreased generation of NADPH and therefore reduced glutathione which protects against oxidant injury by participating as a cofactor in the rxns that neutralize compounds such as H2O2

older cells unable to tolerate oxidative stress

Causes:
drugs- sulfa, primaquine (malaria drugs)
infections
foods

Heinz bodies
Bite cells

begins 2-3 days after exposure to oxidants

Splenomegaly and cholelithiasis are ABSENT

A

G6PD deficiency

X-linked!!!

Both intravascular and extravascular hemolysis

Protect against malaria by increasing the clearance and decreasing the adherence of infected red cells, possibly by raised levels of oxidant stress and causing membrane damage in the parasite-bearing cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what is the genetic defect in sickle cell disease

what does this mutation promote

A

substitution of valine for glutamic acid at position 6 of the beta chain

Mutation promotes the polymerization of deoxygenated hemoglobin, leading to red cell distortion, hemolytic anemia, microvascular obstruction and ischemic tissue damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

sickle cell trait

A

8-10% of African Americans are heterozygous for HbS (offspring of 2 heterozygotes has ¼ chance of having sickle cell disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

sickle cell disease

A

almost all of the Hgb in red cell is HbS (SS)

90% HbS
8% HbF
2% HbA
less severe if HbF remains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

how is the definitive diagnosis of sickle cell made

A

hemoglobin electrophoresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

when do heterozygotes sickle?

A

-Heterozygotes only sickle under conditions of profound hypoxia

43
Q

what is the effect of HbA on sickling

A

-HbA→ interferes with HbS polymerization

44
Q

what is the effect of HbF on sickling

A

-HbF→ inhibits polymerization of HbS even more than HbA so infants aren’t symptomatic until 5-6 months when the level of HbF falls. Hereditary persistence of HbF = less severe sickling

45
Q

HbS and HbC compound heterozygotes

A

(HbSC disease) have symptomatic sickling disorder but milder than sickle cell disease (Than straight SS)

46
Q

increase in MCHC has what effect on sickling

decrease in MCHC?

A

Mean cell hemoglobin concentration
→ increase in MCHC (dehydration) = facilitates sickling
→ decrease in MCHC = reduce disease severity (individual with HbS homozygote AND alpha thalassemia)

47
Q

effect of intracellular pH on sickling

A

-decrease pH→ reduce O2 affinity of hemoglobin → increase deoxygenated HbS→ more sickling

48
Q

transit time has what effect on sickling

A

Transmit time of red cells through microvascular beds

SLOW = more sickling (bone marrow, spleen, inflamed vascular beds)

49
Q

Morphology in sickle cell disease

A

Sickled cells
Reticulocytosis
Target cells
Howell-Jolly bodies

50
Q

how does sickle cell protect against malaria

A

intracellular parasites consume O2 and decrease intracellular pH→ promotes hemoglobin sickling in AS red cells. These cells are stiff and distorted and may be cleared more rapidly by phagocytes in the spleen, liver, keeping parasite load down

51
Q

by what 2 mechanisms do microvascular occlusions occur in sickle cell disease

A

inflammation** and vasoconstriction play a role.
Inflammatory states produced mediators that up-regulate adhesion molecules on endothelial cells→ more likely that sickled red cell gets stuck

Depletion of NO** due to inactivation by free hemoglobin released from lysed sickled red cells

52
Q

what are vaso-occlusive crises

what organs systems are involved

how do pt’s present
(children, males?)

A

aka pain crises, are episodes of hypoxic injury and infarction that cause severe pain in the affected region

o infection, dehydration and acidosis all favor sickling and can act as a trigger

o involves bones, lungs, liver, brain, spleen and penis

• in children → manifests as hand-foot syndrome or dactylitis of hands and/or feet
o Priapism: hypoxic damage and erectile dysfunction

o loss of visual acuity –retinopathy

o stroke→ adhesion of sickle red cells to arterial vascular endothelium and vasoconstriction due to depletion of NO by free hemoglobin

o crippling

53
Q 
chest pain
SOB
lung infiltrates
often precipitated by pneumonia
most common cause of death in adult pt's with sickle cell disease
A

• Acute chest syndrome: vaso-occlusive crisis involving the lungs

54
Q

occur in children with intact spleens with sickle cell disease

Massive entrapment of sickled red cells leads to rapid splenic enlargement, hypovolemia, and sometimes shock. Requires transfusions

A

sequestration crises

55
Q

what disease are associated with aplastic crises

A

sickle cell disease

hereditary spherocytosis

56
Q

what are the effects of chronic sickle cell disease

A

→ impairment of growth and development and organ damage affecting the spleen, heart, kidneys, and lungs.

o Hypertonicity in the renal medulla provokes sickling→ leads to hyposthenuria (inability to concentrate urine) → even more dehydration

57
Q

what are sickle cell pt’s susceptible to ?

A

infection with encapsulated organisms due to altered spleen fn: . Pneumococcus pneumoniae and Haemophilus influenzae septicemia and meningitis

b/c of autosplenectomy

58
Q

disorder of decreased platelet survival- platelet functions are normal

caused by autoantibody mediated destruction of platelets

F>M (Women less than 40 years old)

splenomegaly and LAD are uncommon!

how do you treat?

A

Chronic immune thrombocytopenic purpura

treatment–> splenectomy

platelets are destroyed in the spleen after being coated with antibodies to platelet membrane glycoproteins IIb/IIIa or Ib-IX

spleen is the source of the antibodies and the site of destruction!

59
Q

IgG antibodies active at 37C

splenomegaly

A

warm antibody type

60
Q

IgM antibodies active below 37C

IgM bindings–> agglutinates red cells–> fixes complement (C3b) –> phagocytosis by spleen, liver, bone marrow ]]

appears sometimes after infection (mycoplasma pneumoniae, EBV-mono, influenza virus, cytomegalovirus , HIV)

pallor, cyanosis, Raynaud phenomenon

A

Cold agglutin type

15-30% of cases

61
Q

meningococcemia
rickettsioses

Vitamin C deficiency

henoch-schonlein purpura

Hereditary-Hemorrhagic telangiectasia

Perivascular amyloidosis

platelet number and function normal
PT and PTT normal

A

Bleeding disorders caused by vessel wall abnormalities

62
Q

Cold agglutinin immunohemolytic anemia can be seen with lymphoid neoplasms and infections such as what

A 
Mycoplasma 
EBV-mono
Cytomegalovirus
Influenza virus
HIV
63
Q

small, round inclusions in RBC’s that appear when the spleen is absent

A

howell jolly bodies

64
Q

teardrop cells

A

myelofibrosis and other infiltrative disorders of the marrow

65
Q

macro-ovalocytes

A

seen in megaloblastic anemias - such as Vitamin B12 deficiency

66
Q

what are the causes of warm antibody type immunohemolytic anemia

A

idiopathic –> 50%

predisposing condition (SLE, lymphoid neoplasm)

drug exposure (alpha-methyldopa, penicillin, cephalosporin)

67
Q

positive coombs test

A

presence of anti-RBC antibodie s in the serum and on the surface of RBC

68
Q

ristocetin dependent bioassay that is low

A

vWF deficiency

most cases its autosomal dominant

69
Q

how do you treat vWF deficiency

A

demopressin - stimulates vWF release

infusions of plasma concentrates containing factor VIII and vWF

70
Q

hydroxyurea therapy in sickle cell disease has what benefits/outcomes?

A

inhibitor of DNA synthesis –> increases HbF levels and has anti-inflammatory effect

HbF interferes with HbS polymerization

71
Q

x-linked disorder

10% of African American males

manifests after –> primaquine, sulfonamides, nitrofurantoin, phenacetin, aspirin

heinz bodies

A

G6PD deficiency

72
Q

increased ferritin

reduced TIBC

A

anemia of chronic disease

IL-6 promotes sequestration of storage iron with poor use for EPO

EPO secretion is impaired

73
Q

what are the causes of aplastic anemia

A

Acquired

  • idiopathic- 65%***
  • immune mediated

Chemical agents

  • benzene
  • chemotherapy agents
  • chloramphenicol
  • gold salts

physical agents

  • viral infections
  • whole body irradiation

inherited

  • Fanconi anemia
  • telomerase defects
74
Q

autosomal recessive

defects in multiprotein complex required for DNA repair

evident early in life

usually present with hypoplasia of kidney, spleen, bone anomalies (thumbs)

A

Fanconi anemia (cause of aplastic anemia)

75
Q

abnormally short telomeres

A

found in half of pt’s with aplastic anemia

76
Q

hypocellular bone marrow
many fat cells
fibrous stroma
scattered lymphocytes and plasma cells

dry tap

abnormal bleeding

occurs at any age M=F
insidious
pancytopenia

no splenomegaly

A

Aplastic anemia

77
Q

what is the treatment for aplastic anemia and what is the prognosis

A

BM transplant - 5yr survival of more than 75%

immunosuppressive therapy

78
Q

fever
thrombocytopenia
microangiopathic hemolytic anemia (schistocytes)
transient neurologic deficits (headaches, photophobia, disorientated)
renal failure (elevated creatinine)

A

thrombotic thrombocytopenic purpura

deficiency in plasma enzyme ADAMTS13 (vWF metalloprotease) –> normally degrades very high molecular weight multimers of vWF but in its absence these multimers accumulate in plasma –> platelet activation/aggregation

heart, brain and kidney are most often affected

79
Q

PIGA gene

CD55
CD59
C8 binding protein

X-linked

A

paroxysmal nocturnal hemoglobinuria

*** only hemolytic anemia caused by acquired genetic defect

mutation in PIGA–> PIGA is an enzyme that is essential for the synthesis of certain membrane-associated complement regulatory proteins

PNH blood cells are deficient in GPI-linked proteins that regulate complement activity

1) Decay-accelerating factor (CD55)
2) Membrane inhibitor of reactive lysis (CD59) **most important b/c this is a potent inhibitor of C3 convertase that prevents spontaneous activation of alt. complement pathway
3) C8 binding protein

80
Q

what is the mechanism of injury in PNH

A

Mechanism of injury:
Red cells deficient in
←GPI-linked proteins are susceptible to lysis by complement → manifests as intracellular hemolysis (due to C5-C9 MAC)

**nocturnal portion of disease is due to slight decrease in blood pH during sleep → increases the activity of complement

81
Q

what is the leading cause of death in PNH

what other problem can develop/occur in PNH

A

thrombosis

-40% have venous thrombosis (hepatic, portal, cerebral veins)

5-10% develop AML or myelodysplastic syndrome

82
Q

how do you diagnose PNH

A

flow cytometry

83
Q

how do you treat PNH

A

Eculizumab = monoclonal antibody that prevents conversion of C5 to C5a- lowers risk of thrombosis and reduces hemolysis
Adverse effects: increased risk of serious meningococcal infections

84
Q

A 42-year-old woman has had nosebleeds, easy bruising, and increased bleeding with her menstrual periods for the
past 4 months. On physical examination, her temperature is 37°C, pulse is 88/min, respirations are 18/min, and blood
pressure is 90/60 mm Hg. She has scattered petechiae over the distal extremities. There is no organomegaly. Laboratory
studies show hemoglobin of 12.3 g/dL, hematocrit of 37%, platelet count of 21,500/mm3, and WBC count of 7370/mm3. A
bone marrow biopsy specimen shows a marked increase in megakaryocytes. The prothrombin and partial thromboplastin
times are within the reference range. What is the most likely diagnosis?

A

idiopathic thrombocytopenic purpura

85
Q

where is Vit B12 absorbed

A

terminal ileum

86
Q

Causes:
1) associated with defects in complement factor H
membrane cofactor protein (CD46) or factor I

these are all important in preventing excess activation of the alternative complement pathway

2) acquired antibodies

remitting relapsing course

prominence of renal failure

PT and PTT normal

A

Atypical hemolytic uremic syndrome

87
Q

associated with infectious gastroenteritis caused by E.coli strain O157;H7→ elaborates shiga-like toxin which alters endothelial cell function resulting in platelet activation

  • children and older adults
  • bloody diarrhea
  • supportive care
  • irreversible renal damage and death can occur
A

typical HUS

88
Q

TNF

A

implicated in DIC occurring with sepsis

induces endothelial cells to express TF

decreased thrombomodulin

upregulates expression of adhesion molecules on endothelial cells

89
Q

A 17-year-old boy reports passage of dark urine to his physician. He has a history of multiple bacterial infections and
venous thromboses for the past 10 years, including portal vein thrombosis in the previous year. On physical examination,
his right leg is swollen and tender. CBC shows hemoglobin, 9.8 g/dL; hematocrit, 29.9%; MCV, 92 μm3; platelet count,
150,000/mm3; and WBC count, 3800/mm3 with 24% segmented neutrophils, 1% bands, 64% lymphocytes, 10%
monocytes, and 1% eosinophils. He has a reticulocytosis, and his serum haptoglobin level is very low. A mutation affecting
which of the following gene products is most likely to give rise to this clinical condition?

□ (A) Spectrin
□ (B) Glucose-6-phosphate dehydrogenase
□ (C) Phosphatidylinositol glycan A (PIGA)
□ (D) β-Globin chain
□ (E) Factor V
□ (F) Prothrombin G20210A
A

□ (C) Phosphatidylinositol glycan A (PIGA)

A mutation in this gene prevents the membrane expression of
certain proteins that require a glycolipid anchor. These include proteins that protect cells from lysis by spontaneously
activated complement. As a result, RBCs, granulocytes, and platelets are exquisitely sensitive to the lytic activity of
complement. The RBC lysis is intravascular; patients can have hemoglobinuria (dark urine). Defects in platelet function
are believed to be responsible for venous thrombosis.

90
Q

Laboratory studies show hemoglobin of 8.8 g/dL, hematocrit of 26.3%, platelet count of 199,000/mm3, and
WBC count of 5350/mm3.

α-Globin inclusions are present in erythroblasts and erythrocytes, leading to increased
phagocytosis by cells of the mononuclear phagocyte system.

A

B-thalassemia

91
Q

form of marrow failure in which space occupying lesion replaces normal marrow elements

most common causes:
-metastatic cancer (breast, lung, prostate)

can be a component of the spent phase of myeloproliferative disorders

A

Myelophthisic anemia

marrow distorition and fibrosis

abnormal release of nucleated erythroid precursors and immature granulocytic forms (leukoerythroblastosis)

tear drop shaped red cells (deformed during escape from the fibrotic marrow)

92
Q

Esophageal webs
Microcytic hypochromic anemia
Atrophic glossitis

A

Pulmmr -Vinson syndrome

chronic Iron deficiency anemia

93
Q

stillbirths

A

thalassemia major

94
Q

caused by autoantibodies to platelets (decreased platelet survival)

disease of childhood

M=F

followed viral illness

treatment with glucocorticoids

A

Acute immune thrombocytopenia purpura

95
Q

A 77-year-old man has experienced increasing malaise and a 6-kg weight loss over the past year. He has noted more
severe and constant back pain for the past 3 months. On physical examination, his temperature is 38.7°C. His prostate is
firm and irregular when palpated on digital rectal examination. There is no organomegaly. A stool sample is negative for
occult blood. Laboratory studies include a urine culture positive for Escherichia coli, serum glucose of 70 mg/dL,
creatinine of 1.1 mg/dL, total bilirubin of 1 mg/dL, alkaline phosphatase of 293 U/L, calcium of 10.3 mg/dL, phosphorus of
2.6 mg/dL, and PSA of 25 ng/mL. CBC shows hemoglobin, 9.1 g/dL; hematocrit, 27.3%; MCV, 94 μm3; platelet count,
55,600/mm3; and WBC count, 3570/mm3 with 18% segmented neutrophils, 7% bands, 2% metamyelocytes, 1%
myelocytes, 61% lymphocytes, 11% monocytes, and 3 nucleated RBCs per 100 WBCs. What is the most likely diagnosis?
□ (A) Anemia of chronic disease
□ (B) Aplastic anemia
□ (C) Hemolytic anemia
□ (D) Megaloblastic anemia
□ (E) Myelophthisic anemia
□ (F) Thalassemia

A

(E) This patient has findings most suggestive of prostatic adenocarcinoma that has metastasized to the bone. High
alkaline phosphatase, hypercalcemia, and a leukoerythroblastic pattern in the peripheral blood (immature WBCs and
RBCs) are a consequence of the tumor acting as a space-occupying lesion. Myelophthisic anemias also may be caused
by infections.

96
Q

what are the effects of having SLE hematologically

A

low haptoglobin b/c SLE is an autoimmune disease that can result in hemolysis by means of autoantibodies directed at RBC’s and the Coombs test is often positive

97
Q

interleukin-1, tumor necrosis factor, and
interferon-γ

low EPO
low serum iron
high ferritin
reduced TIBC

A

These cytokines promote sequestration of iron in storage compartments and depress erythropoietin

anemia of chronic disease
-most common cause of anemia in hospitalized pt’s in US

98
Q

IL-6

A

stimulates increase in hepatic production of hepcidin

99
Q

leukoerythroblastosis,

A

Metastases are space-occupying lesions (myelophthisic process)

100
Q

The absence of ADAMTS-13 …

A

The absence of ADAMTS-13 gives rise to large multimers of vWF that promote widespread platelet aggregation,
and the resulting microvascular occlusions in brain, kidney, and elsewhere produce organ dysfunction, thrombocytopenia,
microangiopathic hemolytic anemia (MAHA), and bleeding.

101
Q

deffective aggregation

-auto recessive

  • fail to aggregate in response to ADP, collagen, epi, thrombin b/c of deficiency of glycoprotein IIb-IIIa→ an integrin that participates in “bridge formation” b/w platelets by binding fibrinogen
  • severe bleeding
A

Glanzmann thrombasthenia

102
Q

Hepcidin levels increase when

A

iron stores are high.

103
Q

excessive iron absorption

palpable spleen tip

high serum ferritin

A

beta-thal

104
Q

what can PNH develop into

A

AML

myelodysplastic syndrome

Heme/Lymph II Exam II (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions