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Medical Pathology > Hematologic Disorders II > Flashcards

Hematologic Disorders II Flashcards

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USMLE® Step 1 flashcards
1
Q

What % does anemia show an ocular manifestation

A

10% of pts

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2
Q

in what % of pts do anemia and thrombocytopenia shows ocular manifestation

A

40-70%

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3
Q

what are some signs of ocular manifestations of anemia

A

conjunctival pallor ( if RBC count is 50% of normal); anemia retinopathy

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4
Q

what is characteristic of Anemic retinopathy

A

flame shaped or dot blot hemorrhages, cotton wool spot, Roths spots

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5
Q

what is an increase in the RBC mass

A

polycythemia

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6
Q

which type of polycythemia has loss of vascular fluid

A

relative polycythemia

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7
Q

which type of polycythemia has proliferative disease of the bone marrow ( neoplastic); absolute decrease in total rbc mass; elevated white cell and platelet counts

A

primary polycythemia

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8
Q

which type of polycythemia has increased EPO levels caused by hypoxic conditions ( chronic heart and lung disease, oxygen chamber, and high altitude training)

A

secondary polycythemia

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9
Q

in this condition the pt is at an elevated risk for thrombotic events; 50% increased risk of acute myeloid leukemia in 20 yrs

A

polycythemia

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10
Q

this occurs due to a mutation at JAK2 locus in 95% of cases ( RBC hyperproliferation in bone marrow); Dx by blood test and Tx by bloodletting, AA, Jakafi

A

polycythemia

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11
Q

these are inherited disorders affecting Hb formation

A

Hemoglobinopathies

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12
Q

what are some inherited disorders affecting Hb formation

A

Sickle Cell Anemias ( SS, SC, CC, AC,AS)

Thalassemia

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13
Q

this trait is common in 8% of African americans; usually asymptomatic due to one good “A” gene; confers fitness benefit against malarial infections

A

Sickle cell trait

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14
Q

this is the most common inherited blood disorder ; normocytic- normochromic anemia; autosomal recessive; Abnormal Hb Synthesis; 70k cases in the US; more common in people of African and Hispanic Descent

A

Sickle Cell Anemia

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15
Q

signs of this disorder include hemolytic anemia, intermittent painful crises, multiple organ damage ( spleen, GI, brain, Heart)

A

sickle cell anemia

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16
Q

this disorder caused by a single pt mutation in the beta globulin gene; stress causes sickling ( viral illness, dehydration, hypoxia, exertion); effects are microvascular occlusion and hemolysis

A

sickle cell anemia

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17
Q

what are the five stages of sickle cell retinopathy

A 
1.peripheral arteriolar occlusions
2 peripheral AV anastomoses
3. sea fan neovascularization
4. vitreous hemorrhage
5. traction or rhegmatogenous retinal detachment
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18
Q

which version of sickle cell is high risk for retinopathy

A

SC; SS is worse systemically

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19
Q

this disorder is caused by absence or reduced globin chains in Hb

A

Thalassemias

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20
Q

which type of thalassemia is caused by unstable globin chains that damage the RBC membrane > hemolysis

A

microcytic hypochromic anemia

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21
Q

what are the two types of thalassemia

A

beta ( major) and alpha

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22
Q

this type of thalassemia is severe, fatal in childhood, and common around the mediterranean

A

beta thalassemia

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23
Q

this type of thalassemia is less severe; more common in S Asian and W African nations

A

Alpha thalassemia

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24
Q

what are some causes of excessive bleeding

A

vitamin K def., liver disease, coagulation cascade, platelet abnormality, and meds such as Warfarin, ASA, etc

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25
Q

what are lower than normal levels of platelets in the blood

A

thrombocytopenia

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26
Q

what are some causes of thrombocytopenia

A

pregnancy, autoimmunity, sepsis, TTP & hemolytic uremic syndrome, medications, toxins, cancer, and hypersplenism

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27
Q

signs of this disorder include profuse bleeding during Sx or after dental work; spontaneous bleeding from gums or nose; easy or excessive bruising; prlonged bleeding from cuts; petechiae; blood in urine or stools; heavy menstrual flows

A

thrombocytopenia

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28
Q

how do you Dx thrombocytopnia

A

blood draw; can be treated with transfusion/immunosuppression/splenectomy

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29
Q

this condition is increased risk of bleeding; X linked ; Tx with replacement therapy via infusions

A

Hemophilia

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30
Q

what are the two main types of hemophilia

A

Hemophilia A ( Factor VIII def) and Hemophilia B ( Factor IX def)

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31
Q

what are some ocular manifestations of hemophilia

A

hemorrhages throughout orbit, globe, and adnexa

32
Q

this is the most common inherited bleeding disorder; males = femles ; 1/100 to 1/1000 affected

A

Von willebrand disease

33
Q

what is the pathogenesis of von willebrand disease

A

von willebrand factor def. ( protein required for platelet adhesion)-> can be combined with factor VIII def-> poor thrombus formation due to low platelet aggregration

34
Q

the effects of this disease include varying severity of coagulation def.

A

von willebrand disease

35
Q

this is an autoimmune disorder ( primary and secondary); largely idiopathic ; pathogenesis includes inhibition of coagulation cascade regulatory proteins

A

Antiphospholipid syndrome

36
Q

hypercoagulability is common in this disorder caused by anti-cardiolipin Ab, Anti beta 2 GP Ab, and lupus anticoagulant

A

Antiphospholipid syndrome

37
Q

complications of this disorder include DVT, stroke, recurrent spontaneous abortion. Dx’d with blood work and Tx is blood thinners

A

Antiphospholipid syndrome

38
Q

T or F: Antiphospholipid syndrome seen in women who have had multiple miscarriages

A

T

39
Q

this is one of the most common thrombotic retinal events in hypercoagulable states

A

CRVO

40
Q

what are some quantitative disorders of leukocyte function

A

leukocytosis and leukopenia

41
Q

this is when you have leukoctye counts higher than normal ; pathologic; normal physiologic response to stressors

A

leukocytosis

42
Q

this is when you have leukocyte counts lower than normal; abnormal; low wbc count predisposes to infection

A

leukopenia

43
Q

what is a qualitative disorder

A

disruption of cellular function

44
Q

this is uncontrolled proliferation of malignant leukocytes

A

leukocytosis

45
Q

what is the pathogenesis of leukocytosis

A

single progenitor cell undergoes malignant transformation-> overcrowding of bone marrow -> decreased production and function of normal hematopoietic cells

46
Q

what are the two types of leukemias

A

acute vs. chronic ( myeloid cells vs. Lymphoid cells)

47
Q

what are some manifestations of leukemia

A

petechiae, purpura, and ecchymosis

48
Q

signs of this disorder include anemia, infection, weight loss, bone pain, elevated uric acid, liver, spleen and lymph node enlargement and bleeding

A

leukemias

49
Q

in this type of leukemia , you have undifferentiated or immature cells; rapid onset;

A

acute leukemias

50
Q

which type of acute leukemia ; mean onset is 14 YO; most common childhood leukemia; 80% survival rate at 5 YO and 30-40% in adults

A

acute lymphoblastic leukemia

51
Q

which type of acute leukemia ; most common adult leukemia ( mean age 67; treat with chemo

A

acute myelogenous leukemia

52
Q

in this type of leukemia, mature cells do not function normally ; slow progression

A

chronic leukemias

53
Q

this type of chronic leukemia is usuallyed Dx in adults; tx with bone marrow transplant

A

chronic myelogenous leukemia

54
Q

this type of chronic leukemia has most Dxed over age 55; 70% asymptomatic at Dx; lymphadenopathy; suppression of humoral immunity > increased infection with encapsulated bacteria

A

chronic lymphocytic leukemia

55
Q

T or F: Eye Signs occur in 50-70% of cases of leukemias

A

T ( initial presentation may related to visual complaint; ocular infiltrates assoc with a poor prognosis

56
Q

what are some ophthalmoscopic findings with leukemia

A

retinal infiltrates ( roth Spots, cotton wool spots); ONH edema

57
Q

what are some causes of lymphadenopathy

A

neoplastic disease, immunologic or inflammatory conditions; endocrine disorders; lipid storage disorders

58
Q

what indicates lymphoproliferative disease

A

enlarged spleen

59
Q

when we are palpating for conjunctivitis , where will we feel ?

A

the glands by the ears

60
Q

in this condition, you get painless tumors of the lymph nodes ( can be cervical, axillary, and inguinal);

A

Hodgkins Lymphoma

61
Q

what is the most common presenting symptom of Hodgkins Lymphoma

A

nontender, palpable lymphadenopathy in the neck; contiguous nodes

62
Q

what are some symptoms of hodgkins

A

fever, night sweats, fatigue, unexplained weight loss

63
Q

how is Hodgkins confirmed

A

by lymph node biopsy ; characterized by Reed- Sternberg cells

64
Q

this condition has a higher incidence and is more common than Hodgkins; No reed Sternberg cells; more likely to affect noncontiguous lymph nodes; poorer prognosis than HL

A

Non Hodgkins Lymphoma

65
Q

Are there ocular manifestations more in Hodgkins or Non Hodgkins

A

Non Hodgkins has higher incidence; but rare; 50% of 1 degree orbital malignancies ( majority of the time its in the orbit , small amt in the conjunctiva)

66
Q

how are ocular manifestation of lymphoma differentially Dx’d

A

squamous papilloma, pyogenic granuloma, and lymphangiectasis; Dx’d via biopsy; Tx with beam radiation

67
Q

T or F: pts with Sjogrens are 46 X more likely to develop Non Hodgkins lymphoma than healthy pts

A

T

68
Q

T or F: 5-8% of all pts with Sjogrens Syndrome will develop non Hodgkins Lymphoma

A

T

69
Q

this is the most common primary bone cancer; occurs more in elderly adults ; males more common

A

multiple myeloma

70
Q

in this condition you get the malignant proliferation of plasma cells in bone marrow ;

A

multiple myeloma

71
Q

what are nodular masses in bone narrow asso with multiple myeloma

A

plasmacytomas

72
Q

what happens when you have immature hypergammaglobulinemia assoc with multiple myeloma

A

increased infection due to abnormal immunologlobulin production

73
Q

what happens when you have increased risk of closts /ischemia leading to paraproteinemia

A

monoclonal gammopathy

74
Q

what are some symptoms of multiple myeloma

A

fractures, bone pain, Hypercalcemia, anemia, recurrent infections, renal failure

75
Q

how is multiple myeloma Dx’d

A

punched out bone lesions, monoclonal proteins in plasma or urine

76
Q

how do we treat multiple myeloma

A

high dose chemo and allogenic bone marrow transplantation

Medical Pathology (11 decks)

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