Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

A1 > Hematology > Flashcards

Hematology Flashcards

(200 cards)

Start Studying
1
Q

What ethnicity most commonly has hemophilia C?

A

Ashkenazi Jews

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

What is the function of vWF?

A

facilitates attachment and aggregation of platelets, forming a plug

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

What is the inheritance pattern of hemophilia A? B?

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

What is the MOA of heparin?

A

activates antithrombin

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

What is the MOA of argatroban, bivalirudin, and dabigatran?

A

Direct thrombin inhibitors (factor II)

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

What is MOA of aminocaproic acid?

A

Inhibits tPA

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

What is the MAO of tPA and other thrombolytics?

A

Inhibition of the conversion of plasminogen to plasmin

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

What does PTT measure? PT?

A 
PTT = intrinsic
PT = extrinsic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Which is prolonged PT or PTT with hemophilia A? B?

A

Both PTT

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

Describe the mixing study for screening for hemophilia?

A

Add in normal blood to patient’s blood. If blood clots, (since factors are now present), this strongly suggests hemophilia

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

What factor levels indicate the need for an immediate transfusion of platelets for hemophilia? What about for DDAVP?

A

Less than 1%

More than 5% = DDAVP

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

What is the inheritance pattern of vWF disease?

A

AD

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

What is the most common inherited bleeding disorder?

A

vWF disease

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

What factor levels are decreased in vWF disease?

A

VIII

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

What is the result of a ristocetin cofactor assay with vWF disease?

A

Decreased

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

What is the treatment for vWF disease?

A

DDAVP

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

What will coagulation studies show with vWF disease?

A

Increased bleedings time (from platelet dysfunction) and increased PTT (from loss of factor VIII)

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

What is the pathophysiology of HIT (heparin induced thrombocytopenia)

A

Administration of heparin induces the body to form antibodies to platelets, causing a paradoxical hypercoagulable state

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

What is antiphospholipid antibody syndrome associated with?

A

SLE

RA

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

Hospitalized patient on a heparin drip who develops thrombocytopenia should be suspicious for what disorder?

A

HIT

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

What is the treatment for HIT?

A
  • D/c heparin

- Start argatroban or lepirudin

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

What must women with factor V leiden avoid?

A

OCPs

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

What is the pathophysiology DIC?

A

Deposition of fibrin in small blood vessels, leading to activation of the coag pathways, causing thrombosis and end-organ damage

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

What is the acute presentation of DIC?

A
  • Bleeding from venipuncture sites

- Ecchymoses and petechiae

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What is the chronic presentation of DIC?
- Bruising and bleeding - thrombophlebitis - Renal dysfunction
26
What is the treatment of DIC?
Transfuse RBCs, platelets, and manage shock
27
What is the major difference between DIC and liver disease in terms of coag results?
Unlike liver disease, factor VIII is depressed
28
What is the pathophysiology of thrombotic thrombocytopenic purpura (TTP)?
Deficiency of vWF enzyme (ADAMS-13) results in abnormally large vWF multimers, that aggregate platelets and crease platelet microthrombi
29
What are the five major s/sx of TTP?
- Thrombocytopenia - microangiopathic hemolytic anemia - Neurological changes - Impaired renal function - Fever
30
What will a PBS show with TTP?
Schistocytes
31
What is the pathophysiology of HUS?
E.coli O157:H7 infection causes renal dysfunction, resulting in uremia and thus platelet dysfunction.
32
What is the treatment of TTP?
Plasma exchange | Steroids
33
Why are platelet transfusions contraindicated in TTP?
Additional platelets will be consumed and further the activation cascade
34
What is the pathophysiology of idiopathic thrombocytopenic purpura?
IgG antibodies form against the patient's platelets, cause splenic destruction
35
What diseases are associated with ITP?
SLE HIV CA HCV
36
What is the usual presentation of ITP?
Asymptomatic
37
What is the acute presentation of ITP?
Abrupt onset of hemorrhagic complications following a viral illness
38
What is the chronic presentation of ITP?
Insidious onset of symptoms or incidental thrombocytopenia
39
True or false: ITP is a diagnosis of exclusion
True
40
What is the treatment for ITP?
If platelets more than 30,000, nothing If not, then corticosteroids or IVIG
41
What is the treatment for symptomatic ITP when pharmacotherapy fails?
Splenectomy
42
What are the PE findings of Fe deficiency anemia?
Glossitis Cheilosis Koilonychia
43
What is the role of ferritin?
Stores of Fe in the body
44
What does a large RDW indicate?
Irregular erythropoiesis
45
What is the treatment for Fe deficiency anemia?
Replace Fe orally for 4-6 months.
46
What are the four major causes of microcytic anemias?
- Fe deficiency - Thalassemias - Chronic disease - Sideroblastic anemia
47
What happens to ferritin levels in anemia of chronic disease?
Goes up, but serum Fe stores go down
48
What happens to TIBC in anemia of chronic disease?
Decreases
49
What happens to TIBC in Fe deficiency anemia?
Increases
50
What happens to RDW with Fe deficiency anemia vs anemia of chronic disease?
Increases with Fe deficient Normal with ACD
51
What happens to serum transferrin receptors with Fe deficiency anemia vs anemia of chronic disease?
Increase with Fe deficiency Normal in ACD
52
What are the drugs/chemicals that can cause sideroblastic anemia?
- INH - Amphenicol - Pb - EtOH
53
What is the pathophysiology of sideroblastic anemia?
Inhibition of heme synthesis causes build up of Fe around the nucleus of cells
54
How can you differentiate folate vs B12 deficiency?
Folate deficiency will have only elevated homocysteine, whereas B12 has both elevated levels of homocysteine, and methylmalonic acid
55
What are the bone marrow findings of megaloblastic anemias?
Hypersegmented PMNs
56
What are the steps of the Schilling test?
measures the absorption of cobalamin via ingestion of radiolabeled cobalamin with and without intrinsic factor. The patient is given an unlabeled B12 IM shot to saturate B12 receptors in the liver and an oral challenge of radiolabeled B12. The radiolabeled B12 will pass into the urine if properly absorbed
57
What is the fish tapeworm notorious for causing B12 deficiency?
Diphyllobothrium latum
58
What is the function of haptoglobin, and what happens to levels of it with hemolytic anemia?
Binds free Fe in the blood. Decreased levels in hemolytic anemia 2/2 binding of now free Fe
59
What are the PBS findings of G6PD deficiency?
Bite cells | Heinz bodies
60
What is the pathogenesis of PNH?
PNH is a deficiency in GPI-anchor molecules that keep CD55/CD59 attached to cells, resulting in hemolysis by complement.
61
What is the best diagnostic modality to detect PNH?
CD55/CD59 flow cytometry
62
What is the pathogenesis of hereditary spherocytosis?
Autosomal dominant defect or deficiency in spectrin or ankyrin,
63
What is the best diagnostic modality to detect hereditary spherocytosis?
Osmotic fragility test
64
What type of anemia is had with hereditary spherocytosis?
Extravascular hemolytic anemia
65
What is the treatment for hereditary spherocytosis?
Splenectomy
66
What is the inheritance pattern of sickle cell disease?
AR
67
What are the three major vaccinations that should be given prophylactically to asplenic patients?
Pneumovax HiB Meningococcal
68
What extremity findings may be present in children with sickle cell disease?
dactylitis
69
What organ dysfunction can be caused by sickle cell?
Autosplenectomy Renal Lungs
70
What is acute chest syndrome?
a vaso-occlusive crisis of the pulmonary vasculature commonly seen in patients with sickle cell anemia. This condition commonly manifests with pulmonary infiltrate on a chest x-ray, and is preceded by a lung infection
71
What virus classically causes an aplastic crisis with sickle cell disease?
Parvovirus B19
72
What is the treatment for acute chest syndrome?
Abx | Blood transfusion
73
What are the PBS findings associated with sickle cell? (2)
- Sickle cells | - Howell-Jolly bodies (purple inclusion bodies)
74
What is the reasoning behind using hydroxyurea in sickle cell disease?
Increases HbF production, leading to less sickling
75
What antibody type is associated with warm and cold hemolytic anemia respectively?
``` Warm = IgG Cold = IgM ```
76
What can cause warm autoimmune hemolytic anemia?
SLE | CLL
77
What can cause cold autoimmune hemolytic anemia?
Mycoplasma pneumoniae Mono
78
What is the diagnostic modality of choice for autoimmune hemolytic anemia?
Direct coomb's test
79
What is the treatment for severe warm autoimmune hemolytic anemia?
Steroids
80
What is the MOA of rituximab?
Anti-CD20 antibody
81
Both AIHA and hereditary spherocytosis can present with spherocytes and positive osmotic fragility tests, but only (____) will have a ⊕ direct Coombs test.
AHIA
82
Which has four and which two genes: beta or alpha parts of Hb?
``` Alpha = 4 Beta = 2 ```
83
What ethnicities usually develop thalassemias?
African Middle eastern Asians
84
What will Fe studies show with thalassemias?
Normal
85
What is the drug given to patients who require chronic transfusions to prevent cardiomyopathy?
Deferoxamine
86
What is the treatment for beta thalassemia major and HbH disease?
Chronic transfusions
87
What is the usual presentation of 0/4 alpha globin?
Hydrops fetalis
88
What is HbH disease?
1/4 alpha globin genes Patients have chronic hemolysis, with increased reticulocyte counts, as well as skeletal changes
89
What is alpha thalassemia trait?
2/4 alpha genes | Usually asymptomatic
90
What is the presentation of 3/4 alpha globin genes?
Silent carrier
91
What is the genetic change that causes polycythemia major?
JAK2 mutation (Y-kinase)
92
What happens to EPO with PCV?
Decreased
93
What is the treatment for PCV?
Hydroxyurea and IFN
94
What is the telltale sign of porphyria?
Pink urine
95
What are the 5 Ps of AIP?
- Painful abdomen - Port wine urine - Polyneuropathy - Psych - Precipitated by drugs
96
What is the best way to diagnose AIP? (2)
Urine plasma and porphyrin levels Specific mutated enzyme assay
97
What is the treatment for an acute attack of AIP?
High dose glucose to decrease heme synthesis during attacks (negative feedback)
98
What is the pathophysiology of febrile nonhemolytic transfusion reaction?
Cytokine formation during storage of blood
99
What is the pathophysiology of allergic transfusion reaction?
AB formation against donor proteins
100
What is the pathophysiology of hemolytic transfusion reaction?
Preformed antibodies against donor erythrocytes
101
What is the presentation of febrile nonhemolytic transfusion reaction?
Fever, chills, and malaise 1-6 hours post transfusion
102
What is the presentation of allergic transfusion reaction?
Prominent urticaria
103
What is the presentation of hemolytic transfusion reaction?
Anaphylaxis
104
What is the treatment for hemolytic transfusion reaction?
IVFs
105
What is the treatment for febrile nonhemolytic transfusion reaction?
acetaminophen
106
What is the treatment for allergic transfusion reaction?
Antihistamines
107
What are auer rods, and in which diseases are they seen?
Crystallized myeloblastic protein (MPO) AML
108
What is the most common childhood malignancy?
ALL
109
What is the usual presentation of ALL?
Rapid onset of anemia, thrombocytopenia etc
110
What are the usual PE findings of ALL?
HSM | Bleeding ums
111
What is the treatment to prevent renal damage from AML (besides adequate hydration)?
allopurinol | Rasburicase
112
What, generally, is CLL?
A malignant proliferation of crappy lymphocytes
113
What are the usual s/sx of CLL?
B symptoms Infections HSM
114
Smudge cells are seen in which malignancy?
CLL
115
Which have MPO: myeloblasts or lymphoblasts?
Myeloblasts
116
Which has prominent nucleoli, and which have inconspicuous: myeloblasts vs lymphoblasts?
prominent in myeloblasts Inconspicuous in lymphoblasts
117
What are the sequale of infiltration of bone marrow from CLL?
- Granulocytopenia - Thrombocytopenia - Hypogammaglobulinemia
118
What is the treatment for CLL? Prognosis?
Fludarabine and chlorambucil Used to alleviate symptoms.
119
What are the usual s/sx of CML?
Anemia Splenomegaly B symptoms
120
What are the three phases of CML?
1. Chronic (several years) 2. Accelerated (transition) 3. Blast crisis (acute leukemia phase, few months left)
121
What is the translocation the produces CML?
t(9;22)
122
What is the treatment for CML?
imatinib
123
What are the granulocytes?
PMNs Eosinophils Basophils
124
What is the usual age of presentation for: ALL?
Less than 13
125
What is the usual age of presentation for: AML?
13-40 years
126
What is the usual age of presentation for: CML?
40-60
127
What is the usual age of presentation for: CLL?
60+
128
How can you differentiate a leukemoid reaction from CML?
LAP is high is leukemoid reaction LAP is low in CML
129
What is hairy cell leukemia?
Malignant proliferation of B cells
130
What protein does hairy cell leukemia usually produce?
TRAP
131
What is the treatment for hairy cell leukemia?
Cladribine
132
What is the characteristics cell fro HL? What are the CD markers for this?
Reed-sternberg cells CD15 and CD30
133
What cell line is usually involved with NHL?
Mainly B cell, but sometimes T cells
134
What is the difference between NHL and HL in terms of presentation?
HL = Many peripheral nodes involved NHL = Single group of localized nodes spread contiguously
135
What is the age distribution for NHL?
65-75 year olds
136
What is the age distribution for HL?
Bimodal
137
What is the infectious associated for HL and NHL?
``` HL = EBV NHL = HIV, autoimmune ```
138
What is the natural history of follicular lymphoma?
Indolent course | Painless waxing and waning adenopathy
139
What is the natural h/o diffuse large B cell lymphoma?
Intermediate grade | Single rapidly growin mass
140
What is the treatment regimen for diffuse large B cell lymphoma? Prognosis?
R-CHOP | Good prognosis
141
What are the histological characteristics of Burkitt's lymphoma?
"Starry sky"
142
What infection is associated with Burkitt's lymphoma?
EBV
143
What is the CD marker that is positive in Mantle cell lymphoma?
CD5 (although it is a B cell lymphoma)
144
What is the natural history of adult T cell lymphoma?
HIgh grade--can progress to ALL
145
What is the viral etiology of adult T cell lymphoma?
HTLV
146
What is mycosis fungoides / sezary syndrome?
T cell lymphoma of the skin, that presents with cutaneous lesions and pruritus
147
Skin bx of mycosis fungoides usually shows what?
Cerebriform lymphoid cells
148
What is the gold standard for diagnosing lymphomas?
Excisional lymph node bx
149
What is the treatment for lymphomas in general?
if low grade, palliative | If high, aggressive
150
HL is generally a B or T cell malignancy?
B cell
151
Above what anatomical landmark do HLs usually present?
Above the diaphragm
152
What are the Pel-Ebstein fevers associated with HL?
Alternating febrile/nonfebrile fevers that last 1-2 weeks
153
What are the three major factors of staging for HL?
- Number of lymph nodes - Presence of B symptoms - If above and below diaphragm are involved
154
What subtype of HL has the best prognosis?
Lymphocyte predominant
155
What causes the hypercalcemia associated with MM?
Osteoclast activation with resulting lytic bone lesions
156
Why are patients with MM immunocompromised?
Shoddy, monoclonal IgG and/or IgA production
157
What are the characteristic findings of a serum protein electrophoresis?
Monoclonal spike
158
What is the classic PBS findings with MM?
Rouleaux formation
159
What happens to the total protein:albumin ratio with MM?
Increased
160
What are the characteristics urinary findings in MM?
Bence jones proteins
161
What is the treatment for young patients with MM?
Autologous bone marrow transplant
162
What is the treatment for old patients with MM?
Melphalan (oral alkylating agent) and prednisone
163
What causes adult fanconi syndrome?
MM damaging renal tubules
164
M protein spike = ?
Waldenstrom's macroglobulinemia
165
What, generally, is Waldenstrom's macroglobulinemia?
Clonal disorder of B cells that leads to a malignant monoclonal gammopathy--INcreased levels of IgM
166
What are the hemodynamic changes that can occur with Waldenstrom's macroglobulinemia?
Hyperviscosity syndrome
167
What are the usual s/sx of Waldenstrom's macroglobulinemia?
- Raynaud phenomenon - Neuro problems - Blurred vision 2/2 engorged blood vessels
168
What can MGUS progress to?
Waldenstrom's | MM
169
What is the dolg standard for diagnosing Waldenstrom's macroglobulinemia? What will this show?
Bone marrow bx Dutcher bodies (PAS+ IgM deposits around the nucleus)
170
What is the treatment for Waldenstrom's?
Chemo for the underlying malignancy Plasmapheresis to remove excess immunoglobin
171
What is the cause of AL amyloidosis?
Plasma cell dyscrasia with deposition of monoclonal light chain fragments
172
What is the cause of AA amyloidosis?
Deposition of the acute phase reactant serum amyloid A | e.g. chronic inflammatory diseases
173
What is the cause of dialysis related amyloidosis?
Beta-2 microglobulin deposits
174
What is the cause of Heritable amyloidosis?
Deposition of abnormal gene products (prealbumin)
175
What is the senile-systemic amyloidosis?
Deposition of otherwise normal transthyretin
176
What are the the three major organs that are affected by amyloidosis?
Kidneys Heart Liver
177
What is the diagnostic test of choice for amyloidosis?
Bx with congo red stain
178
What is the treatment for primary amyloidosis?
Experimental chemo | autologous stem cell transplant
179
What is the technical definition of neutropenia?
ANC less than 1500
180
What is the most common cause of neutropenia?
infections followed by drugs
181
What is the usual presentation of chronic neutropenia?
Recurrent sinusitis Gingivitis Perianal infections
182
What lab tests should be ordered in the work up of neutropenia?
ANA levels
183
What is the treatment for neutropenia?
ABx G-CSF IVIG
184
What is the treatment for neutropenic fever?
Broad spectrum abx--cefepime
185
What are the components of the NAACP mnemonic for eosinophilia?
- Neoplasm - Allergies - Asthma - Collagen vascular dz - Parasites
186
CSF analysis showing eosinophilia is suggestive of what?
of a drug reaction or infection with coccidioidomycosis or a helminth.
187
Hematuria + eosinophilia may be ?
Schistosomiasis
188
What is a syngeneic transplant?
From identical twin
189
What is the cause of GVHD?
Minor histocompatibility complex antigens
190
What are the s/sx of GVHD?
- Skin changes - Cholestatic liver dysfunction - obstructive lung path
191
Down syndrome patients are predisposed to what neoplastic process?
ALL
192
What condition is associated with SCC and BCC of the skin?
Xeroderma pigmentosum
193
What condition is associated with malignant melanomas?
Multiple dysplastic nevi
194
What cancer is associated with chronic atrophic gastritis?
Gastric adenocarcinoma
195
What cancer is associated with ulcerative colitis?
Colonic adenocarcinoma
196
What cancer is associated with tuberous sclerosis?
AStrocytoma and cardiac rhabdomyomas
197
What cancer is associated with Paget's disease of the bone?
osteosarcoma and fibrosarcoma
198
What cancer is associated with Plummer vinson syndrome?
SCC of the esophagus
199
What cancer is associated with autoimmune disease?
Malignant thymomas
200
What is the treatment for an acute transplant rejection?
- Corticosteroids - Antilymphocyte abs - Tacrolimus

A1 (32 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions