Abnormal Bleeding

The parents of a 13-month-old boy are contacted by a local social agency because the day
care they are using has reported them for

suspected child abuse. The abuse was suspected because the day care personnel thought
that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the chairman of
the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have multiple
large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object such as a
belt buckle or rod) are seen. A blood smear isunremarkable. PTT is prolonged, and PT
and bleeding times are normaI.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Acute myelogenous leukemia
/ B. Disseminated intravascular coagulation
/ C. Hemophilia
/ D. Thrombocytopenia
/ E. Von Willebrand's disease
Explanation - Q: 1.1
Close
The correct answer is C. This child has hemophilia, which is an X-linked

recessive disorder of clotting. The severity of the symptoms experienced can
vary with the degree of enzymatic block in the production of the affected
clotting factor. Unfortunately, some cases may reach medical attention only
when the child begins to experience large numbers of falls when learning to
walk, and the parents are suspected of child abuse. The severity of the
symptoms can vary markedly from case to case, since even as little as 2-5%
of the normal levels of the missing clotting factor can markedly reduce
symptoms. Symptoms can include bruising, spontaneous bleeding, bleeding
into joints with associated pain and swelling, gastrointestinal and urinary tract
hemorrhage, and prolonged bleeding from cuts, tooth extractions, or surgery.
Acute myelogenous leukemia (choice A) and thrombocytopenia (choice D)
can also produce a tendency to bruise, but would not be associated with
normal peripheral smears.
Disseminated intravascular coagulation (choice B) is usually seen in patients
with other severe diseases, such as sepsis or complicated pregnancy.
Von Willebrand's disease (choice E) tends to cause oozing blood after injury
rather than bruising, and is associated with a long bleeding time.
The parents of a 13-month-old boy are contacted by a local social agency

because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.
Question 2 of 6
Deficiency of which of the following has been linked to this patient's condition?
/A. Factor VII
/B. Factor VIII
/C. Factor X
/D. Factor XII
/E. Factor XIII
Close
The correct answer is B. Hemophilia can be caused by a deficiency of either

factor VIII (classic hemophilia or hemophilia A) or factor IX (Christmas
disease or hemophilia B). The two variations are very similar clinically,
although the appropriate factor must be replaced when treating an acute
bleeding episode. Both of these factors are in the intrinsic coagulation
pathway. The other factors listed in the choices are not involved.
Question 3 of 6
This child's condition is due to a defective enzyme encoded on which of the

following chromosomes?
/A. 13
/B. 18
/C. 21
/D. X
/E. Y
Close
The correct answer is D. Both the hemophilia A and hemophilia B genes are
located on the X chromosome. While almost all affected individuals are boys
(rare exceptions are girls with a carrier mother and an affected father), the Y
chromosome (choice E) is not involved.
Associate chromosome 13 (choice A) with the trisomy known as Patau
syndrome.
Associate chromosome 18 (choice B) with the trisomy known as Edwards
syndrome.
Associate chromosome 21 (choice C) with the trisomy known as Down
syndrome.
Question 4 of 6
Which of the following can temporarily increase secretion of the needed factor in
some patients with a milder form of this disease?
/A. Desmopressin
/B. Epsilon-aminocaproic acid

/C. Factor VIII concentrate
/D. Factor IX concentrate
/E. Fresh frozen plasma
Close
The correct answer is A. Desmopressin can temporarily raise factor VIII

levels in a patient with mild classic hemophilia by causing the release of
factor VIII stored in the body. The use of desmopressin before dental work or
small surgical procedures may allow such procedures to be performed
without using factor VIII concentrates.
Epsilon-aminocaproic acid (choice B) is a anti-fibrinolytic agent used to slow
the degradation of blood clots, particularly in patients with hemophilia B.
Fresh frozen plasma (choice E) contains both factor VIII (choice C) and
factor IX (choice D), both of which are also available as concentrates. These
products are used to raise clotting factor levels, usually in patients who are
either actively bleeding or who are expected to be actively bleeding because
of surgery or dental procedures.
Question 5 of 6
The patient continues to experience persistent bleeding. Which of the following

would be the most appropriate pharmacotherapy?
/A. Aminocaproic acid
/B. Aspirin
/C. Desmopressin
/D. High-dose IV immune globulin
/E. Prednisone
Close
The correct answer is A. Aminocaproic acid is a synthetic hemostatic with a
specific antifibrinolysis action. The medication inhibits plasminogen activator

substance, which is concerned with the destruction of clots. This medication
does not control bleeding caused by a loss of vascular integrity. Aminocaproic
acid is indicated for control of excessive bleeding resulting from systemic
hyperfibrinolysis, well as for prevention of bleeding in hemophiliacs
undergoing a surgical procedure. It is contraindicated for use in patients with
severe renal impairment or active disseminated intravascular clotting.
Aspirin (choice B) is absolutely contraindicated for use in this patient since it
would result in a more severe bleed.
Desmopressin is a synthetic analog of arginine vasopressin (choice C), the
naturally occurring human antidiuretic hormone (ADH). This agent is
generally used only in milder forms of the disease.
High-dose IV immune globulin (choice D) is most commonly used in the
treatment of ITP (idiopathic thrombocytopenic purpura) to rapidly raise the
platelet count.
Prednisone (choice E) is a glucocorticoid indicated for the treatment of
idiopathic thrombocytopenic purpura (IV dosing only) as well as in the
treatment of a variety of inflammatory conditions. It would be ineffective in this
patient.
Question 6 of 6
In approximately what year did effective screening of blood products begin to

limit the transmission of HIV and hepatitis B in these patients?
/A. 1955
/B. 1965
/C. 1975
/D. 1985
/E. 1995
Close
The correct answer is D. This is clinically important information, because

hemophiliac children who were diagnosed after 1985 have had very little
chance of developing AIDS or hepatitis as a complication of their disease
(they may still get exposures by other routes, e.g., sexual), while these were
major problems in patients who were treated earlier. Fortunately, recently
developed recombinant DNA factor VIII products are coming into increasing
use, and offer the chance of providing truly adequate hemostatic control
without any increased viral risk.
Following a viral illness, a previously healthy 3-year-old child develops multiple
petechiae and purpura, and is noted to be oozing blood from
the mouth. Prothrombin and activated partial thromboplastin times are within
normal limits. Review of a peripheral smear demonstrates that
platelet counts are markedly decreased, but all the platelets that are present
appear morphologically normaI. The remainder of the blood
smear and additional bone marrow studies are normaI.
Question 1 of 4

/A. Disseminated intravascular coagulation
/B. Hemophilia A
/C. Hemophilia B
/D. Idiopathic thrombocytopenic purpura
/E. Von Willebrand disease
Close
The correct answer is D. Idiopathic thrombocytopenic purpura (ITP), also

known as immune thrombocytopenic purpura, now that the immunologic
basis of the disease has been better defined, is a hemorrhagic disorder that
can present in two very different forms in children and adults. Children (peak
ages 2-4 years) tend to present with acute, self-limited thrombocytopenia that
often follows a viral (or immunization) trigger. In contrast, adults (peak ages
20-50 years) tend to have an insidious development of thrombocytopenia that
may persist for long periods. In both types of ITP, the hemorrhagic diathesis is
the result of a pure thrombocytopenia that does not affect prothrombin (PT)
and activated partial thromboplastin (PTT) times because the blood clotting
factors are present in normal quantities.
In disseminated intravascular coagulation (choice A), platelets are often
decreased as a result of consumption, but PT and PTT are prolonged.
In hemophilia A and B (choices B and C), platelets are present in normal
numbers, PT is normal, and PTT is long.

In von Willebrand disease (choice E), platelet counts are normal, but platelet
function is decreased because of decreased or abnormal von Willebrand
factor.
Question 2 of 4
Which of the following tests would be most helpful in confirming the probable
diagnosis?
/A. D-dimer levels
/B. Factor VIII concentration
/C. Factor IX concentration
/D. IgG autoantibodies on platelet surfaces
/E. Total plasma von Willebrand factor antigen
Close
The correct answer is D. While ITP is mostly a diagnosis of exclusion, the

presence of IgG autoantibodies on the platelet surface can be an important
confirmatory test result.
High D-dimer levels (choice A) suggest disseminated intravascular
coagulation.
Low factor VIII (choice B) suggests hemophilia A.
Low factor IX (choice C) suggests hemophilia B.
Low or abnormal von Willebrand factor antigen (choice E) suggests von
Willebrand disease.

Question 3 of 4
Most patients with this condition have antibodies to which of the following?
/A. PIatelet ADP
/B. PIatelet membrane cholesterol
/C. PIatelet membrane glycoproteins
/D. PIatelet membrane lipids
/E. PIatelet thromboxane A2
Close
The correct answer is C. The autoantibodies are usually directed against

platelet membrane glycoproteins.
ADP (choice A) and thromboxane A2 (choice E) are involved in platelet
aggregation.
Cholesterol (choice B) and lipids (choice D) are components of virtually all
mammalian plasma membranes.
Question 4 of 4
Approximately what percentage of children with this condition will undergo

spontaneous remission?
/A. Less than 5%
/B. 15%
/C. 30%
/D. 60%
/E. More than 80%
Close
The correct answer is E. More than 80% of childhood cases of ITP undergo
spontaneous remission, which frequently occurs within 2 months.
Spontaneous remission is uncommon (2%) in adults, but more than 60% will
eventually recover with treatment. Treatment in children is controversial;
treatment in adults can involve splenectomy, glucocorticoids, and intravenous
IgG. Approximately 1% of children with ITP and 5% of adults with ITP die from
hemorrhagic complications, including intracranial hemorrhage.
A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.
Question 1 of 5
The fact the abdomen is rigid with guarding suggests which of the following?
/A. Colon cancer
/B. Diverticulitis
/C. Liver failure
/D. Peritonitis
/E. Shock
Close
The correct answer is D. The usual reason for a patient to have a rigid
abdomen is that peritonitis is present, and is causing severe pain related to
peritoneal nerve fiber stimulation. The probable cause of the peritonitis is a
ruptured appendix. This patient is also probably in shock (choice E), as
indicated by the hypotension with increased respirations and heart rate, but
this would not cause the abdominal guarding. The other answers are
distracters.
Question 2 of 5
Given this patient's presentation, which of the following is the most likely cause of
his petechiae, purpura, and mucosal blood oozing?
/
A. Disseminated intravascular coagulation
/B. Hemophilia A
/C. Idiopathic thrombocytopenic purpura
/D. Von Willebrand disease
/E. Wiskott-AIdrich syndrome
Close
The correct answer is A. You should suspect disseminated intravascular

coagulation (DIC) in patients who are seriously ill with other disease who then
develop evidence of a coagulopathy. In this case, sepsis is the probable
inciting event. DIC is thought to occur in 30-50% of patients with sepsis, and
may, in many cases, be triggered by a reaction to gram-negative or
staphylococcal cell wall material. Other settings in which DIC can be a
complication include major trauma, obstetric complications, acute myelocytic
leukemias, disseminated carcinomas, burns, massive transfusions, acute
hepatic failure, myocardial infarction, and inflammatory conditions (e.g.,
ulcerative colitis, rheumatoid arthritis, Crohn disease, sarcoidosis).
The other conditions listed in the choices would not be expected to be
specifically associated with sepsis, although for medical/legal and safety
reasons, supporting diagnostic tests should be ordered to exclude previously
undiagnosed disease.
Hemophilia (choice B) is a hereditary coagulopathy with X-linked genetics
that typically presents in early childhood with extensive tissue hemorrhages
and hemoarthroses following minor trauma.
Idiopathic thrombocytopenic purpura (choice C) is an acquired autoimmune
disease characterized, in adults, by chronic coagulopathy with petechiae,
purpura, and mucosal bleeding. It is not usually associated with any other
systemic diseases, such as this patient's appendicitis and probable sepsis.
Von Willebrand disease (choice D) is hereditary, with usually autosomal
dominant genetics. It typically causes prolonged menstrual bleeding and
prolonged bleeding from small cuts and surgical procedures. Petechiae and
ecchymoses are less often seen.
Wiskott-Aldrich syndrome (choice E) is a hereditary disease with X-linked
genetics, thrombocytopenia, eczema, and recurrent infection that tends to
present in children with bloody diarrhea.

Question 3 of 5
Which of the following findings on screening laboratory tests would be most

consistent with the probable diagnosis?
/A. Decreased platelet count, increased prothrombin time, increased activated
partial thromboplastin time
/B. Increased platelet count, increased prothrombin time, normal activated
/C. Increased platelet count, normal prothrombin time, increased activated
/D. Normal platelet count, increased prothrombin time, increased activated
/E. Normal platelet count, normal prothrombin time, normal activated partial
thromboplastin time
Close
The correct answer is A. Platelet count is invariably decreased (and usually

obvious on peripheral smear) in DIC, and this is one of the more reliable
screening tests for the condition (although it does not exclude many other
diagnoses). Both prothrombin time and activated partial thromboplastin times
are often prolonged, but you should be aware that they might each be
shortened or normal as well in DIC. Thus, these two commonly performed
tests are actually not as useful as they might appear. The other choices listed
are wrong because the platelet counts are not decreased.
Question 4 of 5
For confirmation of the probable diagnosis, the emergency department physician

orders a D-dimer test. D-dimer is which of the following?
/A. A clotting factor in the common coagulation pathway

/B. A clotting factor in the extrinsic coagulation pathway
/C. A clotting factor in the intrinsic coagulation pathway
/D. A factor found in the complement pathway
/E. A fibrin degradation product
Close
The correct answer is E. A variety of specialized tests have been developed

to help in the diagnosis of disseminated intravascular coagulation.
Unfortunately, many of these are only available in major medical centers or
through reference laboratories, which markedly limits their usefulness in
emergency rooms elsewhere. One of the more available and useful of these
tests is the D-dimer tests, which measures a fibrin degradation product that is
made when a cross-linked clot (indicating the presence of thrombin to form
the clots) is lysed by plasmin. One way to understand disseminated
intravascular coagulation is to think of it as primarily a thrombotic problem,
with excessive clot formation and then lysis, which leads secondarily to a
hemorrhagic problem as platelets and clotting factors are consumed. High Ddimer levels indicate that clots are forming and then lysing at an unusual rate
in the body, and thus, together with a low platelet count, suggest that DIC is
present. Functionally, DIC can be subdivided into acute and chronic
presentations, which tend to occur in somewhat different settings. In the
acute presentations, the hemorrhagic processes often dominate the clinical
difficulties, while in the chronic presentations, the clotting tendencies often
dominate the picture. The D-dimers are not in themselves factors (e.g.,
substances to be consumed) in either the coagulation or complement
pathways.
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Question 5 of 5
Which of the following would be most effective in diminishing the rate at which
clot formation is occurring?
/A. Aminocaproic acid
/B. Heparin
/C. PIatelet transfusion
/D. Red cell transfusion
/E.
Tranexamic acid
Close
The correct answer is B. The therapy of disseminated intravascular

coagulation is complex and problematic, and, frankly, not always successful.
The patients are often already critically ill, and the DIC they have developed
often contributes to major organ failure. The basic problem in therapy is how
to treat simultaneous bleeding and clotting tendencies. Paradoxically, while
the bleeding tendency may be appearing to dominate the clinical picture,
treatment with the anticoagulant heparin may actually help the patient by
decreasing the rate at which the clotting factors are being consumed. This
must, of course, be done very carefully, since an "overshoot" may exacerbate
the patient's problems. Blood components including platelet transfusions
(choice C), red cell transfusions (choice D), and cryoprecipitate are also
sometimes used to restore the blood to a closer to normal state. As a last
resort when other measures have failed, some physicians will also try
antifibrinolytic agents such as aminocaproic acid (choice A) and tranexamic
acid (choice E) to try to keep clots from dissolving and exacerbating the
bleeding tendency.
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A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.

The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 1 of 5

/A. Disseminated intravascular coagulation
/B. Hemophilia A
/C. Hemophilia B
/D. Idiopathic thrombocytopenic purpura
/E. Von Willebrand disease
The correct answer is E. Von Willebrand disease is a usually autosomal
Close
dominant condition in which there is either a quantitative or qualitative

abnormality of von Willebrand factor (VWF) production. You should also be
aware that the disease can uncommonly be autosomal recessive or acquired
(associated with Wilms tumor, congenital heart disease, systemic lupus
erythematosus, seizures treated with valproic acid, and hypothyroidism). The
presentation and laboratory findings illustrated are typical. The disease is lifelong, and patients may also present with menorrhagia, post-operative
bleeding, postpartum bleeding, or gingival bleeding. Activated partial
thromboplastin time may be either normal or prolonged, depending on the
degree to which the intrinsic blood coagulation pathway has been inhibited by
decreased binding of factor VIII to von Willebrand factor.
Disseminated intravascular coagulation (choice A) and idiopathic
thrombocytopenic purpura (choice D) are both acquired conditions.
Hemophilia A and B (choice B and C) have X-linked genetics.
Question 2 of 5
Multimers of all sizes of von Willebrand's factor form complexes in plasma with
which of the following?
/A. Factor V
/B. Factor VI
/C. Factor VIII
/D. Factor IX
/E. Factor X
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Close
The correct answer is C. Normal plasma levels of clotting factor VIII are
maintained by complexing with multimers of Von Willebrand factor. This
protects factor VIII from degradation and helps to deliver it to the site of injury.
This is the contribution VWF makes to the intrinsic pathway of coagulation.
The other factors listed do not complex with VWF.
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Question 3 of 5
Very large von Willebrand's factor multimers contribute most to which of the
following platelet activities?
/A. Normal adherence of platelets to damaged blood vessels
/B. Normal budding of platelets from megakaryocytes
/C. Normal digestion of platelets during clot lysis
/D. Normal discharge of granules from platelets
/E. Normal transport of platelets in the blood
Close
The correct answer is A. The typical platelet life span begins in the bone
marrow when platelets bud off from megakaryocytes, and are released to
float freely in the blood stream. When a damaged blood vessel is
encountered, VWF acts as an adhesive bridge between the platelets and the
damaged subendothelium at the site of vascular injury. The platelets
subsequently contract and release granules to help in formation of the clot.
Eventually, the clot lyses, and macrophages consume the platelet debris.
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Question 4 of 5
Which of the following is the binding site for the von Willebrand's factor multimers
on the platelets?
/A. ADP
/B. Factor VIII
/C. GIycoprotein 1b
/D. Thromboxane A2
/E. Prostaglandin l2
Close
The correct answer is C. Von Willebrand factor binds on platelets to the

specific receptor glycoprotein 1b when acting as a bridge between the
platelets and the site of vascular injury.
ADP (choice A) is released by damaged erythrocytes and helps VWF to
adhere to the exposed collagen. It is not, however, the binding factor that
connects the VWF to the platelets.
Factor VIII (choice B) does not participate directly in the platelet portion of
blood clot formation.
Thromboxane A2(choice D) promotes subendothelial exposure after vascular
injury and induces platelet aggregation.
Prostaglandin I2(choice E) inhibits subendothelial exposure after minor
vascular injury.
Question 5 of 5
In an individual with one of the milder forms of the disease presented in this
case, which of the following medications could be used to
increase von Willebrand's factor and factor VIII after treatment?
/A. Aspirin
/B. Aminocaproic acid
/C. Desmopressin
/D. Ibuprofen
/E. Naproxen
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Close
The correct answer is C. Desmopressin is a synthetic analogue of

antidiuretic hormone that can be administered intravenously, intranasally, or
subcutaneously, and is frequently used as the primary treatment for bleeding
in individuals with mild von Willebrand disease. Its function in this disease is
to cause release of VWF from endothelial storage sites, so its use is reserved
for acute treatment of bleeding symptoms (including home treatment with the
high dose intranasal preparation), rather than as a routine medication. Factor
VIII concentrates that also contain VWF in high molecular weight form are
used in the treatment of patients with the more severe forms of Von
Willebrand disease. With treatment, the majority of patients, particularly those
with milder forms of Von Willebrand disease, have normal life spans.
Aminocaproic acid (choice B) is an inhibitor of fibrinolysis that is used to
maintain clots in some individuals with von Willebrand disease.
Drugs with platelet inhibiting activities, including aspirin (choice A), ibuprofen
(choice D), and naproxen (choice E), should be avoided in patients with
disorders of hemostasis.

Abnormal Bleeding

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Abnormal Bleeding

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The parents of a 13-month-old boy are contacted by a local social agency because the day

care they are using has reported them for

The correct answer is C. This child has hemophilia, which is an X-linked

The parents of a 13-month-old boy are contacted by a local social agency

The correct answer is B. Hemophilia can be caused by a deficiency of either

This child's condition is due to a defective enzyme encoded on which of the

/B. Epsilon-aminocaproic acid

The correct answer is A. Desmopressin can temporarily raise factor VIII

The patient continues to experience persistent bleeding. Which of the following

The correct answer is A. Aminocaproic acid is a synthetic hemostatic with a

specific antifibrinolysis action. The medication inhibits plasminogen activator

In approximately what year did effective screening of blood products begin to

The correct answer is D. This is clinically important information, because

Which of the following is the most likely diagnosis?

The correct answer is D. Idiopathic thrombocytopenic purpura (ITP), also

numbers, PT is normal, and PTT is long.

The correct answer is D. While ITP is mostly a diagnosis of exclusion, the

Following a viral illness, a previously healthy 3-year-old child develops multiple

The correct answer is C. The autoantibodies are usually directed against

Approximately what percentage of children with this condition will undergo

The correct answer is A. You should suspect disseminated intravascular

A 23-year-old man is admitted to the hospital through the emergency department

Which of the following findings on screening laboratory tests would be most

The correct answer is A. Platelet count is invariably decreased (and usually

For confirmation of the probable diagnosis, the emergency department physician

/A. A clotting factor in the common coagulation pathway

The correct answer is E. A variety of specialized tests have been developed

A 23-year-old man is admitted to the hospital through the emergency department

The correct answer is B. The therapy of disseminated intravascular

A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.

Which of the following is the most likely diagnosis?

dominant condition in which there is either a quantitative or qualitative

A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.

A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.

The correct answer is C. Von Willebrand factor binds on platelets to the

The correct answer is C. Desmopressin is a synthetic analogue of

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