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Systems Pathology II Final > White Blood Cell Disorders 2 > Flashcards

White Blood Cell Disorders 2 Flashcards

1
Q

What is the most common plasma cell cancer?

A

Multiply Myeloma (MM)

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2
Q

What age group is more commonly affected by multiple myeloma?

A

Older adults (average diagnosis at 70 years)

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3
Q

What gender and race are more likely to have multiple myeloma?

A

Males of African descent

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4
Q

What are the 3 overall features of MM?

A

1 osteolytic lesions
2 neurological issues
3 renal failure

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5
Q

What WBC condition presents with osteolytic “punched-out” lesions?

A

Multiple myeloma

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6
Q

The osteolytic lesions associated with MM lead to elevated serum levels of which mineral?

A

Calcium

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7
Q

What neurological symptoms can be caused by multiple myeloma?

A

Headache, dizziness, tinnitus, confusion

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8
Q

Detection of Bence-Jones proteins may be indicative of what condition?

A

Multiple myeloma

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9
Q

How is MM diagnosed?

A

Multifocal lytic lesions, Bence-Jones proteins in urine, M-spike on protein electrophoresis

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10
Q

What procedure confirms a MM diagnosis?

A

Marrow biopsy

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11
Q

What is the prognosis for MM?

A

Poor: average survival = 4-6 years

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12
Q

What is the treatment for MM?

A

Plasmapheresis and stem cell transplants

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13
Q

What is the most common site of an osteolytic lesions associated with multiple myeloma?

A

Vertebral column at 66% (can also be in ribs, skull, pelvis, femur, clavicle, or scapula)

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14
Q

TINY “punched-out” bony lesions of the cranium can be indicative of what condition but possibly confused with MM?

A

Hyperparathyroidism

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15
Q

What is the hallmark microscopic feature of Hodgkin lymphoma?

A

Reed-Sternberg cells (large cell with owl-eye nuclear appearance)

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16
Q

In what way is the lymphadenopathy of Hodgkin lymphoma similar to follicular lymphoma? How is it different?

A

Both are painless, but Hodgkin lymphoma presents with a single and isolated node

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17
Q

What are the symptoms of Hodgkin lymphoma?

A

Fever, night sweats, anemia, cachexia, splenomegaly, hepatomegaly

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18
Q

What are the risk factors for Hodgkin lymphoma?

A 
Males ages 15-40 and over 55
Family history
History of EBV infection (70%)
Exposure to Agent Orange pesticide
Immunosuppression
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19
Q

If Reed-Sternberg cells are seen under microscopy, what condition can be assumed?

A

Hodgkin lymphoma

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20
Q

What is the prognosis for Hodgkin lymphoma?

A

Favorable with chemotherapy and radiation

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21
Q

Radiation of Hodgkin lymphoma increased the risk of what types of secondary cancers?

A

Lung, melanoma, breast

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22
Q

Myeloid neoplasms most commonly affect what age group?

A

Adults

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23
Q

Myeloid neoplasms arise from transformed myeloblast which are what types of cells?

A

Neutrophils, basophils, eosinophils, erythrocytes, platelets

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24
Q

What are the three categories of myeloid neoplasms?

A

1 acute myelogenous leukemia (AML)
2 myelodysplastic syndromes
3 chronic myeloproliferative disorders

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25
Q

What is the average age of diagnosis of acute myeloid leukemia?

A

50

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26
Q

What is displaced by immature myeloblasts during acute myeloid leukemia?

A

Marrow

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27
Q

What important process is suppressed with acute myeloid leukemia?

A

Hematopoiesis

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28
Q

Acute myeloid leukemia may resemble which other condition?

A

Acute lymphoblastic leukemia

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29
Q

Auer rods upon microscopy are associated with what condition?

A

Acute myeloid leukemia

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30
Q

What is a major feature of AML?

A

Pancytopenia

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31
Q

What is the prognosis for AML and what makes it worse?

A

Poor: 15-30% long term survival; worse with history of myelodysplastic syndromes

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32
Q

What conditions were previously known as “pre-leukemias”?

A

Myelodysplastic syndromes

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33
Q

What is the most common age range of diagnosis for myelodysplastic syndromes?

A

50-70 years

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34
Q

What happens with myelodysplstic syndromes?

A

Marrow fills with myeloblasts and the ability to differentiate cells is disordered

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35
Q

Why were myelodysplastic syndromes previously known as pre-leukemia?

A

40% of MDS will transform into AML

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36
Q

Megaloblasts (hypercellular marrow) seen in what condition will resemble megaloblastic anemia?

A

Myelodysplastic syndromes

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37
Q

What are risk factors for MDS?

A

Chemotherapy, irradiation, monosomy and trisomy

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38
Q

What is the prognosis for MDS?

A

Poor: median of 1-2 years and poor response to treatment of chemo and blood transfusions

39
Q

What are the categories of chronic myeloproliferative disorders?

A

1 chronic myelogenous leukemia (CML)
2 polycythemia vera
3 primary myelofibrosis

40
Q

In what kind of myeloid conditions is the ability to differentiate myeloid stem cells retained but the ratios become disproportionate?

A

Chronic myeloproliferative disorders

41
Q

What is the age range for chronic myelogenous leukemia?

A

25-60 years old

42
Q

Which specific cells are involved with the leukocytosis of chronic myelogenous leukemia?

A

Granulocytes and platelets

43
Q

Extreme splenomegaly is associated with which myeloid condition?

A

Chronic myelogenous leukemia

44
Q

Philadelphia (Ph) chromosome is associated with which condition in 95% of cases?

A

Chronic myelogenous leukemia (CML)

45
Q

What is the medication for chronic myelogenous leukemia?

A

Tyrosine kinase inhibitors

46
Q

What is the accelerated phase of CML called that 50% of all cases reach?

A

“Spent phase” or “blast crisis”

47
Q

What is the result of the Philadelphia chromosome?

A

Decreased myeloblast dependence on GFs

48
Q

The red pulp of the spleen presents as a more beefy and massive appearance in which condition?

A

CML

49
Q

What mutation is associated with polycythemia vera?

A

Point mutations: JAK2 - downstream signal of EPO

50
Q

What is the average age of diagnosis of PCV?

A

60 years (older adults)

51
Q

How is PCV diagnosed?

A

Polycythemia and decreased erythropoietin

52
Q

What is the major concern with PCV?

A

Blood viscosity due to increase of whole blood volume

53
Q

What organs are involved with the multiple organ congestion of PCV that can lead to infarction?

A

Heart, spleen, kidneys

54
Q

What is the prognosis for PCV with and without treatment?

A 
With = 10-20 years post diagnosis
Without = 3 years
55
Q

What condition presents as diffuse marrow fibrosis?

A

Primary myelofibrosis

56
Q

What organ primary enlarges with primary myelofibrosis?

A

Spleen (infarctions are common)

57
Q

What is the appearance of the RBCs in the peripheral blood with primary myelofibrosis?

A

Dacrocytes

58
Q

What are histiocytes?

A

Macrophages or dendritic cells

59
Q

What are the dendritic cells of the skin/mucosa?

A

Langerhans cells

60
Q

Birbeck granules making organelles look like “tennis-rackets” are associated with what condition?

A

Histolytic neoplasms

61
Q

What is the location for unisystem Langerhans cell histiocytosis?

A

Calvary, ribs, femur

62
Q

All forms of unisystem Langerhans cell histiocytosis are associated with what protein abnormality and issue?

A

Abnormal Ras and increased signal transduction

63
Q

Multisystem Langerhans cell histiocytosis is more common among what gender, age group, and race?

A

Males, children under 2 years, and Caucasians

64
Q

What are the signs of Langerhans cell histiocytosis?

A

Fever, multifocal skin lesions

65
Q

What is the prognosis of Langerhans cell histiocytosis?

A

Without treatment, fatal

With chemo, 5 years survival (50%)

66
Q

What are signs of bleeding disorders?

A

Petechiae, ecchymosis

67
Q

What is the stimulus for disseminated intravascular coagulation?

A

Widespread injury (examples = MVA, crush injuries)

68
Q

What kinds of conditions can cause endothelial injury that could progress into DIC?

A

Sepsis, SLE, heat stroke, cancer, birth

69
Q

Thrombocytopenia is common amount what kind of patients?

A

HIV/AIDS

70
Q

What is purpura?

A

Red/purple skin discolorations

71
Q

What causes immune thrombocytopenic purpura?

A

IgG antibodies attack platelets

72
Q

Which form of immune thrombocytopenic purpura affects children? Females 20-40 years of age?

A

Children = acute ITP

Females 20-40 years of age = Chronic

73
Q

What are signs of chronic immune thrombocytopenic purpura?

A

Petechiae, epistaxis, gum bleeding, easy bruising

74
Q

What is the treatment for immune thrombocytopenic purpura that leads to 65% remission?

A

Splenctomy

75
Q

What is the inheritance pattern for von Willebrand Disease?

A

Autosomal dominant

76
Q

What is the result of decreased von Willebrand factor?

A

Dysfunctional platelet adherence to vessel wall

77
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease (1% of the population)

78
Q

Von Willebrand Disease mimics what condition?

A

Thrombocytopenia

79
Q

What is the treatment for von Willebrand Disease?

A

Transfusions or medication to increase vWF (also avoid blood thinners like aspirin and Coumadin)

80
Q

What is the inheritance pattern for hemophilia A?

A

X-linked recessive

81
Q

What gender is most likely to have hemophilia A?

A

Males

82
Q

Which coagulation factor is mutated with hemophilia A? B?

A

Hemophilia A = VIII

Hemophilia B = IX

83
Q

Which is more common: hemophilia A or B?

A

Hemophilia A

84
Q

What condition is also known as Christmas disease?

A

Hemophilia B

85
Q

Which affects soft tissues and joints: hemophilia A or B?

A

A

86
Q

Which blood disorder has the unique feature of absent petechiae?

A

Hemophilia A

87
Q

What is the treatment for hemophilia A and B?

A

Infusion of corresponding coagulation factor

88
Q

What is the inheritance pattern for hemophilia B?

A

X-linked (males more affected)

89
Q

What is unique about the symptomalogy of hemophilia A and B?

A

Clinically identical

90
Q

Thymic hyperplasia is seen in what conditions?

A

Myasthenia gravis, SLE, RA

91
Q

What is an early treatment option for thyme hyperplasia?

A

Thymectomy

92
Q

What causes thymic hyperplasia?

A

Reactive B cells in thymus

93
Q

A thymoma most commonly affects what populations?

A

Adults or myasthenia gravis patients

94
Q

What are the risk factors for developing a thymoma?

A

Asian or EBV infection

Systems Pathology II Final (8 decks)

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