1.
Acute DIC clinical presentation: bleeding predominates
2.
Acute leukemias splenomegaly: moderate splenomegaly
3.
Acute splenic congestion (central venous congestion) splenomegaly: mild splenomegaly
4.
Acute splenitis (blood-borne infection) splenomegaly: mild splenomegaly
5.
Adult patient presents with fever, CNS changes, schistocytes, and thrombotic microangiopathy: thrombotic thrombocytopenic purpura (classic pentad)
6.
Amyloidosis splenomegaly: moderate splenomegaly
7.
Autoimmune hemolytic anemia splenomegaly: moderate splenomegaly
8.
Child presents with renal failure, and lack of CNS changes: hemolytic uremic syndrome
9.
Chronic congestion (portal hypertension, splenic vein obstruction) splenomegaly: moderate splenomegaly
10.
Chronic DIC clinical presentation: thrombosis predominates
11.
Chronic lymphocytic leukemia splenomegaly: severe splenomegaly
12.
Chronic myeloid leukemia splenomegaly: severe splenomegaly
13.
Chronic splenitis due to infective endocarditis splenomegaly: moderate splenomegaly
14.
Describe platelet count, bleeding time, prothrombin time, and partial thromboplastin time in thrombocytopenia.: decreased platelet count
increased bleeding time
normal prothrombin time
normal partial thromboplastin time
15.
Disorder characterized by decreased high molecular weight Von-Willebrand multimers: Type 2 Von-Willebrand disease
16.
Disorder characterized by deficiency of coagulation factor 8: hemophilia A
17.
Disseminated intravascular coagulation bleeding time, platelet count, prothrombin time, partial thromboplastin time: all abnormal
18.
Factor 8 deficiency with moderate symptoms: 1-5%
19.
Factor 8 deficiency with severe symptoms: <1%
20.
Factor 8 fx: activates factor 10 (intrinsic pathway)
21.
Factor associated with hemophilia B: factor 9 deficiency
22.
Granulomatous diseases splenomegaly: moderate splenomegaly
23.
Hairy cell leukemia splenomegaly: severe splenomegaly
24.
Hemophilia A bleeding time, platelet count, prothrombin time, partial thrombplastin time: increased bleeding time
normal platelet count
normal prothrombin time
increased partial thromboplastin time
25.
Hemophilia A pattern of inheritance: X-linked recessive
26.
Hemophilia A Rx: purified or recombinant factor 8
27.
Hemophilia B pattern of inheritance: X-linked recessive
28.
Hereditary spherocytosis splenomegaly: moderate splenomegaly
29.
Idiopathic thrombocytopenic purpura adult sex and age range: females 20-40 years of age
30.
In thymoma, what component of the thymus undergoes neoplastic proliferation?: epithelial component
31.
Infectious mononucleosis splenomegaly: mild splenomegaly
32.
Langerhans histiocytosis splenomegaly: moderate splenomegaly
33.
List 2 autoimmune antibodies created in adults afflicted with idiopathic thrombcytopenic purpura.: anti-platelet membrane Gp 2b/3a
anti-platelet membrane 1b/1x
34.
List 2 autoimmune disorders that lead to destruction of platelets.: Idiopathic thrombocytopenic purpura
Systemic lupus erythematosus: thrombocytopenia is the most common bleeding disorder in SLE.
35.
List 2 binding targets for Von-Willebrand factor.: collagen
platelet membrane GP1b
36.
List 2 components of thrombi in thrombotic microangiopathies.: platelets
fibrin
37.
List 2 consequences of splenomegaly.: hypersplenism
rupture
38.
List 2 end organ dysfunctions that occur in thrombotic microangiopathies.: renal failure
mental status changes
39.
List 2 general causes of decreased platelet production.: bone marrow failure
decreased megakaryocytic in bone marrow
40.
List 2 types of thrombotic microangiopathies.: thrombotic thrombocytopenic purpura
hemolytic uremic syndrome
41.
List 3 autoimmune disorders that present with lymphoid follicles in the thymus (thymic hyperplasia).: Myasthenia gravis: present in 65-75% of cases
Systemic lupus erythematosus
Rheumatoid arthritis
42.
List 3 causes of bone marrow failure.: aplastic anemia
myelophthisic anemia
myelodysplastic disorders
chemotherapy (bone marrow transplant)
drug reactions
infections
43.
List 3 causes of platelet dysfunction.: NSAIDs
Uremia
Vonn Willebrand disease
44.
List 3 drugs that can cause autoimmune destruction of platelets.: quinidine
heparin
sulfa
45.
List 3 general causes of DIC.: obstetric complications
infections
neoplasia (M3)
massive tissue injury
miscellaneous (acute IV hemolysis, snake bites, shock, heat stroke, vasculitis, aortic aneurysm, liver disease)
46.
List 3 viruses that cause infection-associated autoimmune destruction of platelets.: EBV
HIV
CMV
*may also decrease platelet production
47.
List 4 causes of acquired coagulation disorders.: Vitamin K deficiency
Liver disease: most coagulation factors are made in the liver
Massive transfusion
DIC
48.
List 4 general causes of thrombocytopenia.: Decreased production
Increased consumption/destruction
Sequestration (hypersplenism)
Massive transfusion (dilution): transfused blood does not have too many platelets
49.
List a common location of thymomas.: anterior superior mediastinal mass (30-40%)
50.
Long-standing congestion splenomegaly: severe splenomegaly
51.
Lymphomas splenomegaly: severe splenomegaly
52.
Malaria splenomegaly: severe splenomegaly
53.
Metastatic neoplasm splenomegaly: moderate splenomegaly
54.
Mild splenomegaly weight: < 500 g
55.
MMMF splenomegaly: severe splenomegaly
56.
Moderate splenomegaly weight: 500-1000 g
57.
Patient complains of easy bruising and massive bleeding after trauma (hemorrhage into joints as well).: hemophilia A
58.
Patient presents with a cough, shortness of breath, and superior vena cava syndrome: thymoma in anterior superior mediastinal compartment
59.
Patient presents with normal PTT and prolonged PT.: factor 7 deficiency
60.
Patient presents with petechiae, ecchymoses, epistaxis, mucosal bleeding and CNS hemorrhages: thrombocytopenia
*CNS hemorrhages are the most severe complication of low platelet counts.
61.
Platelet dysfunction bleeding time, platelet count, prothrombin time, partial thrombplastin time: increased bleeding time, the rest are normal
62.
Platelet dysfunction clinical features (3): petechiae
ecchymosis
excessive bleeding
63.
Post-traumatic bleeding platelet count: < 50,000/micro liters
64.
Review clinical course of DIC.: 
65.
Review morphology of DIC.: 
66.
Review pathophysiology of DIC.: 
*In thrombotic thrombocytopenic purpura, only platelets are consumed; DIC consumes both platelets and coagulation factors. 12
67.
Review platelet clotting.: 
68.
Severe splenomegaly weight: > 1000 g
69.
Spontaneous bleeding platelet count: < 20,000/microliter
70.
Thalassemia major splenomegaly: moderate splenomegaly
71.
Thombotic microangiopathy associated with endothelial cell injury due to gastroenteritis caused by E. coli toxin: hemolytic uremic syndrome
72.
Thrombotic microangiopathies RBC morphology: schistocytes
73.
Thrombotic microangiopathy that affects adults: thrombotic thrombocytopenic purpura
74.
Thrombotic microangiopathy that affects children: hemolytic uremic syndrome
75.
Thymoma age group: middle age
76.
Type 1 thymoma morphology and behavior: 
77.
Type 1 Von-Willebrand disease bleeding time, platelet count, prothrombin time, partial thromboplastin time: increased bleeding time
normal platelet count
normal prothrombin time
increased partial thromboplastin time (decreased factor 8)
78.
Type 2 thymoma morphology and behavior:
79.
Von-Willebrand disease pattern of inheritance: autosomal dominant
80.
What 4 coagulation factors are decreased due to vitamin K deficiency?: factor 2
factor 7
factor 9
factor 10
81.
What causes abnormal platelet activation in thrombotic thrombocytopenic purpura?: metalloprotease deficiency (ADAMTS13) <-- either inherited deficiency or autoantibody (most common)
*ADAMTS13 keeps Von-Willebrand factor at a certain size. Absence of the metalloprotease allows VWF to get very large and cause platelet aggregation.
82.
What is the most common cause of acquired coagulation disorder?: vitamin K deficiency
83.
What is the most common hereditary deficiency associated with serious bleeding?: hemophilia A (factor 8 deficiency)
84.
What is the usual cause of idiopathic thrombocytopenic purpura in children?: viral infection
85.
What percent of people with hemophilia A have antibodies against factor 8?: 15%
86.
What systemic disease is associated with thymomas?: myasthenia gravis (30-45%)
*15-20% of myasthenia gravis patients have thymoma
87.
What type of Von-Willebrand disease is associated with decreased circulating Von-Willebrand factor?: type 1 --> secondary decrease in factor 8
88.
What type of Von-Willebrand disease is associated with decreased high molecular weight VWF multimers?: type 2
*opposite of thrombotic thrombocytopenic purpura
89.
Where is factor 8 synthesized?: in the liver
90.
Where is Von-Willebrand factor synthesized?: endothelial cells
91.
Which coagulation has the shortest biological half-life?: factor 7
92.
Which sex is more often afflicted with hemophilia A?: males (X-linked)
93.
Widespread formation of thrombi in microcirculation due to platelet and fibrin deposition.: thrombotic microangiopathies
