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Hematology And Oncology > FA + MTB 2 > Flashcards

FA + MTB 2 Flashcards

1
Q

Hemophilia deficiency and type of inherited

A

Hemophilia A - 8 - XR
Hemophilia B - 9 - XR
Hemophilia C - 11 - AR

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2
Q

Hemophilia A, B, C - treatment

A

A: desmopressin + factor 8 concentrate
B: factor 9 concentrate
C: Factor 11 concentrate

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3
Q

Hemophilia A, B, C - PTT, PT

A

normal PT

increased PTT

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4
Q

Vitamin K deficiency - PT, PTT, BT

A

increased PT, PTT

normal BT

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5
Q

Platelets abnormalities - sign symptoms labs

A
  1. Microhemorrhage (mucus bleeding , epistaxis, petechiae, purpura
  2. Increased bleeding time
  3. Possible decreased platelet count
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6
Q

Bernard-Soulier - PC, BT

A

PC: normal or decreased
BT: increased

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7
Q

Bernard-Soulier diagnosis

A

No agglutination on ristocetin cofactor assay

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8
Q

Bernard-Soulier pathophysiology

A

Deficiency of Gp1b (platelets - vWf adhesion)

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9
Q

Bernard-Soulier findings

A
  1. Defects in platelet plug formation

2. Large platelets (younger)

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10
Q

Glanzmann thrombasthenia - PC,BT

A

PC: -
BT: increased

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11
Q

Glanzmann thrombasthenia pathophysiology

A

GpIIb/IIIa deficiency –> defects in platelet to platelet aggregation

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12
Q

Glanzmann thrombasthenia labs

A

Blood smear shows no platelet climbing

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13
Q

Glanzmann thrombasthenia - agglutination

A

Agglutation with ristocetin

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14
Q

Immune thrombocytopenia pathophysiology

A

Anti GpIIb/IIIa antibodies –> splenic macrophages consumption of platelet antibody complex
Commonly after viral illness

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15
Q

Immune thrombocytopenia labs

A

Increased megakaryocytes on bone bone marrow biopsy

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16
Q

Immune thrombocytopenia treatment

A
  1. Steroids
  2. IV immunoglobulins
  3. splenectomy (for refractory)
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17
Q

Immune thrombocytopenia - PC, BT

A

PC: decreased
BT: increased

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18
Q

Thrombotic thrombocytopenic purpura pathophysiology

A

Inhibition or deficiency of ADAMTS 13 (vwf metalloprotease) –> decreased degradation of vwf multimers –> large vwf multimers –> platelets aggregation and thrombosis

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19
Q

Thrombotic thrombocytopenic purpura labs and symptoms

A

LDH increased
Schistocytes
symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

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20
Q

Thrombotic thrombocytopenic purpura treatment

A
  1. Plasmapheris

2. Steroids

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21
Q

Thrombotic thrombocytopenic purpura - PC, BT

A

PC: decreased
BT: increased

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22
Q

Hemolytic-uremic syndrome - typically seen in/due to

A

children, accompanied by diarrhea and commonly caused by Shiga-toxin-producing E-coli (eg. 157:H7)
in adults does not present with diarrhea
STEC infection is not required

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23
Q

Hemolytic-uremic syndrome - treatment

A

plasmapheresis

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24
Q

Hemolytic-uremic syndrome - symptoms

A

neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

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25
Hemolytic-uremic syndrome - characterised by
1. thrombocytopenia 2. microangiopathic hemolytic anemia 3. acute renal failure
26
Hemolytic-uremic syndrome - PC, BT
decreased PC | increased BT
27
MC inherited bleeding disorder / mode of inheritance
Von willebrand disease (but mild) | AD
28
Von willebrand disease diagnosis / treatment
No agglutination with ristocetin | Desmopressin (release vWF stored in endothelium)
29
Von willebrand disease - PC, BT, PT, PTT
PC normal BT increased PT normal PTT increased or normal
30
DIC - PC, BT, PT, PTT
PC decreased BT increased PT increased PTT increased
31
Causes of DIC - results in
STOP Making New Thrombi Sepsis (gram -), trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion results in widespread activation of clootting --> deficiency of factors --> bleeding state
32
hereditary thrombosis syndromes leading to hypercoagulability - types
1. antithrombin def 2. V leiden 3. protein S or C def 4. Prothrombin gene mutation
33
Prothrombin gene mutation?
Mutation in 3 untranslated region --> increased production of prothrombin --> increased plasma levels and VENOUS thrombosis
34
Factor V Leiden - definition and mechanism
- Production of mutant factor V, resistant to pr C
35
MCC of inherited hypercoagubility in Caucasians
V Leiden
36
Factor V Leiden - complications
1. DVT 2. cerebral vein thromboses 3. recurrent pregnancy loss
37
Protein C or S deficiency pathophysiology
Decreased ability to inactivate Va and VIIIa
38
Protein C or S deficiency increases the risk of
Thrombotic skin and subcutaneous necrosis with hemorrhage following administration of warfarin
39
Causes of Antithrombin deficiency
1. Inherited deficiency | 2. Renal failure/nephrotic syndrome --> loss in urine
40
Antithrombin deficiency pathophysiology
Decreased inhibition of factors 2a and 10a
41
Antithrombin deficiency - PTT, PT, Thrombin time
no direct effects on the PTT, PT, or thrombin time --> diminishes the increase in PTT following heparin
42
Fresh frozen plasma / prothrombin complex concentrate- dosage effect / clinical use
fresh frozen plasma: all coagulation factors + plasma proteins prothrombin complex concentrate: 2, 7, 9, 10, C, S increase coagulation factor levels 1. DIC 2. Cirrhosis 3. immediate warfarin reversal
43
Cryoprecipitate - dosage effect
contains: 1. fibrinogen 2. fibronectin | 3. VWF 4. factor VIII 5. factor XIII
44
Cryoprecipitate - clinical use
coagulation factor deficiencies involving fibrinogen and factor VIII
45
blood transfusion risks
1. infection transmission (low) 2. transfusion reaction 3. iron overload 4. hypocalcemia 5. hyperkalemia
46
Hodgkin - strongly associated with | non-Hodgkin - may be associated with
- EBV - 1. HIV 2. autoimmune diseases
47
Hodgkin lymphoma - men or women
more common in men except for nodular sclerosing type
48
MC Hodgkin lymphoma
Nodular sclerosing
49
Hodgkin lymphoma with best prognosis
lymphocyte-rich
50
Hodgkin lymphoma - seen in immunocrompomised
1. Mixed cellularity | 2. Lymphocyte depleted
51
Non-Hodgkin lymphoma B cells - types
1. Burkitt lymphoma 2. Diffuse large B-cell lymphoma 3. Follicular lymphoma 4. Mantle cell lymphoma 5. Primary central nervous system lymphoma 6. Marginal zone lymphoma
52
Burkitt lymphoma - occurs in (age) / associations
adolescents or young adults | EBV
53
Burkitt lymphoma - genetics
t(8;14) - translocation of c-myc (8) and heavy - chain Ig (14)
54
Burkitt lymphoma - histology
starry sky appearance, sheets of lymphocytes with interspersed "tingible body" macrophages
55
Burkitt lymphoma - forms
``` endemic form (africa) --> jaw sporadic form --> pelvis + abdomen ```
56
MC type of Non-Hodgkin lymphoma
Diffuse large B-cell lymphoma
57
Diffuse large B-cell lymphoma - occurs in (age)
usually older adults but 20% in chidren
58
Diffuse large B-cell lymphoma - genetics
Bcl-2 / Bcl-6 alternations
59
Follicular lymphoma - occurs in (age) / genetics
adults | t(14;18) - translocation of heavy-chain Ig (14) and BCL-2 (18)
60
Follicular lymphoma presents with
painless waxing and waning lymphadenopathy (indolent course)
61
Mantle cells lymphoma - occurs in/ markers/ presentation
older male CD5+ patiens typically present with late stage disease (very aggressive)
62
Mantle cells lymphoma - genetics
t(11:14) - translocation of cycle D1 (11) and heavy-chain Ig (14)
63
Marginal zone lymphoma - definition
neoplastic proliferation of small B cells (cd20) that expands the marginal zone
64
Marginal zone lymphoma - associated with
chronic inflammatory states such as Hashimoto, Sjogren, H. Pylori MALTOMA is marginal zone of mucosal sites
65
1ry CNS lymphoma
adults AIDS/EBV
66
Non-Hodgkin lymphoma T cells - types
1. Adults T-cells lymphoma | 2. Mycosis fungoides/Sezary syndrome
67
Adults T-cells lymphoma is caused by
HTLV (associated with IV drug abuse)
68
Adults T-cells lymphoma - symptoms
1. cutaneous lesions 2, Lytic bone lesions 3. Hypercalcemia
69
Mycosis fungoides present with
skin patches/plaques (cutaneous T-cell lymphoma)
70
Mycosis fungoides - histology
atypical CD4+ cells with cerebriform nuclei
71
Mycosis fungoides may progress to
Sezary syndrome (T-cell leukemia)
72
Multiple myeloma is the most common primary tumor ....
arising with bone in people older than 40-50 | MORE THAN 10% OF BM
73
Multiple myeloma - produces (proportions)
large ammount of: - IgG (55%) - IgA (25%) - Ig light chains
74
Multiple myeloma - symptoms/findings
1. hypercalcemia 2. anemia 3. renal involvement 4. bone lytic lesion/back pain 5. infections 6. Rouleaux formation 7. Bence Jones protein 8. Primary amyloidosis (AL) 9 . M spike on electrophoresis
75
Monoclonal gammopathy of undetermined significance - definition / symptoms / complication
monoclonal expansion of plasma cells (bone marrow less than 10%) asymptomatic may lead to multiple myeloma (1-2% per year)
76
Waldestrom macroblobulinemia - symptoms.mechanism
M spike = IgM --> hyperviscosity syndrome --> 1. blurred vision 2. Raynaud phenomenon
77
Multiple Myeloma - cytokines
high serum Il-6
78
Myelodysplastic syndromes - mechanism
stem cell disorders involving ineffective hematopoiesis | --> defects in cell maturation of all nonlymphoid lineages
79
Myelodysplastic syndromes is caused by
1. de novo mutations | 2. environmental exposure (radiation, benzene, chemotherapy)
80
Myelodysplastic syndromes - complications
may progress to AML
81
Pseudo-Pelger-Huet anomaly
neutrophils with bilobed nuclei typically seen after chemotherapy

Hematology And Oncology (21 decks)

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