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Flashcards in FA + MTB 2 Deck (81):
1

Hemophilia deficiency and type of inherited

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

2

Hemophilia A, B, C - treatment

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

3

Hemophilia A, B, C - PTT, PT

normal PT
increased PTT

4

Vitamin K deficiency - PT, PTT, BT

increased PT, PTT
normal BT

5

Platelets abnormalities - sign symptoms labs

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

6

Bernard-Soulier - PC, BT

PC: normal or decreased
BT: increased

7

Bernard-Soulier diagnosis

No agglutination on ristocetin cofactor assay

8

Bernard-Soulier pathophysiology

Deficiency of Gp1b (platelets - vWf adhesion)

9

Bernard-Soulier findings

1. Defects in platelet plug formation
2. Large platelets (younger)

10

Glanzmann thrombasthenia - PC,BT

PC: -
BT: increased

11

Glanzmann thrombasthenia pathophysiology

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

12

Glanzmann thrombasthenia labs

Blood smear shows no platelet climbing

13

Glanzmann thrombasthenia - agglutination

Agglutation with ristocetin

14

Immune thrombocytopenia pathophysiology

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

15

Immune thrombocytopenia labs

Increased megakaryocytes on bone bone marrow biopsy

16

Immune thrombocytopenia treatment

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

17

Immune thrombocytopenia - PC, BT

PC: decreased
BT: increased

18

Thrombotic thrombocytopenic purpura pathophysiology

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

19

Thrombotic thrombocytopenic purpura labs and symptoms

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

20

Thrombotic thrombocytopenic purpura treatment

1. Plasmapheris
2. Steroids

21

Thrombotic thrombocytopenic purpura - PC, BT

PC: decreased
BT: increased

22

Hemolytic-uremic syndrome - typically seen in/due to

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

23

Hemolytic-uremic syndrome - treatment

plasmapheresis

24

Hemolytic-uremic syndrome - symptoms

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

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