Question 1

An advantage of prenatal diagnosis by genotypic analysis for hemophilia over phenotypic analysis is:

Accepted Answer

A)  ABO blood type does not affect the analysis 
B)  A chorionic villus biopsy can be tested at 4 weeks gestation 
C)  Direct DNA diagnosis is available for all families 
D)  It can be done as a point-of-care test 
A)  ABO blood type does not affect the analysis 
B)  A chorionic villus biopsy can be tested at 4 weeks gestation 
C)  Direct DNA diagnosis is available for all families 
D)  It can be done as a point-of-care test

Question 2

Which assay would assess for the presence of a circulating nonspecific inhibitor?

Accepted Answer

A)  Bethesda titer 
B)  Factor-specific inhibitor 
C)  Lupus anticoagulant 
D)  D-dimer 
A)  Bethesda titer 
B)  Factor-specific inhibitor 
C)  Lupus anticoagulant 
D)  D-dimer

Question 3

Which of the following is used in the identification of a secondary hemostatic pathway anomaly?

Accepted Answer

A)  Bleeding time 
B)  ACE activity 
C)  Platelet aggregation studies 
D)  Factor assays 
A)  Bleeding time 
B)  ACE activity 
C)  Platelet aggregation studies 
D)  Factor assays

Question 4

Explain why disseminated intravascular coagulation is not a primary disease state. Correlate etiology, pathophysiology, and laboratory analysis in your response.

Accepted Answer

Disseminated intravascular coagulation i

Question 5

A 34-year-old woman has just given birth to a healthy baby boy. After the delivery of the baby, the physician proceeds to deliver the placenta. After it has been extracted from the mother's uterus, the resident examines it and notes that it appears incomplete. They try to deliver the remainder but are unsuccessful. A few moments later, the mother loses consciousness, and is bleeding profusely from her uterus. Blood is collected, and the results follow. PT: 17 sec (RR: 11.3-12.8 sec)
INR: 3.02
APTT: 67 sec (RR: 23-32 sec)
D-dimer: 540 ng/mL (RR: <400 ng/mL)
Fibrinogen: 82 mg/dL (RR: 200-400 mg/dL)
Factor V assay: 12%
Based on this information, from what is the most probable diagnosis?

Accepted Answer

A)  Hemophilia A 
B)  von Willebrand's disease 
C)  Placenta previa 
D)  DIC 
A)  Hemophilia A 
B)  von Willebrand's disease 
C)  Placenta previa 
D)  DIC

Question 6

What plasma factor levels of the deficient factor can be expected in a female carrier of FVIII or FIX deficiency?

Accepted Answer

A)  25% of the normal plasma levels 
B)  12.5% of the normal plasma levels 
C)  50% of the normal plasma levels 
D)  5% of the normal plasma levels 
A)  25% of the normal plasma levels 
B)  12.5% of the normal plasma levels 
C)  50% of the normal plasma levels 
D)  5% of the normal plasma levels

Question 7

A patient might be suffering from a coagulopathy. Which of the following physical manifestations would suggest a primary hemostatic pathway problem?

Accepted Answer

A)  Hematomas 
B)  Petechiae 
C)  Joint and muscle bleeding 
D)  Ecchymoses 
A)  Hematomas 
B)  Petechiae 
C)  Joint and muscle bleeding 
D)  Ecchymoses

Question 8

One of the most common hemostatic abnormalities found in "sick" infants is:

Accepted Answer

A)  DIC 
B)  Factor XIII deficiency 
C)  Hemophilia 
D)  Vitamin K deficiency 
A)  DIC 
B)  Factor XIII deficiency 
C)  Hemophilia 
D)  Vitamin K deficiency

Question 9

What term describes hereditary or acquired disorders that relate to the absence of a coagulation protein or to a protein that is present in the plasma but is functionally defective?

Accepted Answer

A)  Circulating anticoagulant 
B)  Lupus anticoagulant 
C)  Deficiency 
D)  Dysfunctional 
A)  Circulating anticoagulant 
B)  Lupus anticoagulant 
C)  Deficiency 
D)  Dysfunctional

Question 10

Predict results that will confirm the patient's diagnosis.

Accepted Answer

A)  Abnormal aggregation with Ristocetin 
B)  An increased D-dimer 
C)  Decreased activity of either factors VIII or IX 
D)  The presence of an inhibitor pattern in a mixing study 
A)  Abnormal aggregation with Ristocetin 
B)  An increased D-dimer 
C)  Decreased activity of either factors VIII or IX 
D)  The presence of an inhibitor pattern in a mixing study

Question 11

Which is the most common FVIII mutation in patients with a severe phenotype (occurring in almost 50% of patients)?

Accepted Answer

A)  Gross deletion of entire gene locus 
B)  Point mutation involving the thrombin cleavage site 
C)  Inversion mutation of intron 22 
D)  Point mutation involving the site of VWF attachment 
A)  Gross deletion of entire gene locus 
B)  Point mutation involving the thrombin cleavage site 
C)  Inversion mutation of intron 22 
D)  Point mutation involving the site of VWF attachment

Exam 34: Disorders of Secondary Hemostasis

An advantage of prenatal diagnosis by genotypic analysis for hemophilia over phenotypic analysis is:

Which assay would assess for the presence of a circulating nonspecific inhibitor?

Which of the following is used in the identification of a secondary hemostatic pathway anomaly?

Explain why disseminated intravascular coagulation is not a primary disease state. Correlate etiology, pathophysiology, and laboratory analysis in your response.

What plasma factor levels of the deficient factor can be expected in a female carrier of FVIII or FIX deficiency?

A patient might be suffering from a coagulopathy. Which of the following physical manifestations would suggest a primary hemostatic pathway problem?

One of the most common hemostatic abnormalities found in "sick" infants is:

What term describes hereditary or acquired disorders that relate to the absence of a coagulation protein or to a protein that is present in the plasma but is functionally defective?

Predict results that will confirm the patient's diagnosis.

Which is the most common FVIII mutation in patients with a severe phenotype (occurring in almost 50% of patients)?

Introduction

Cellular Homeostasis

Structure and Function of Hematopoietic Organs

Hematopoiesis

The Erythrocyte

Hemoglobin

Granulocytes and Monocytes

Lymphocytes

The Platelet

The Complete Blood Count and Peripheral Blood Smear Evaluation

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopathies: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Hypoproliferative Anemias

Hemolytic Anemia: Membrane Defects

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Disorders of Leukocytes: Granulocytes and Monocytes

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Precursor Lymphoid Neoplasms

Mature Lymphoid Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Thrombophilia

Hemostasis: Laboratory Testing and Instrumentation

Hematology Procedures

Bone Marrow Examination

Automation in Hematology

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Quality Assessment in the Hematology Laboratory

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