Hematologic Malignancies Flashcards

1
Q

Disease that is characterized by a constellation of cytopenias, hypercelluar marrow, morphologic dysplasia, and genetic abnormalities.

A

Myelodysplastic Syndrome

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2
Q

What are the general causes of Myelodysplastic Syndrome (MDS)

A

Usually Idiopathic
Cytotoxic Chemotherapy
Radiation

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3
Q

What is the key distinction of Myelodysplastic Syndrome (MDS)

A

Increase in bone marrow blasts
(immature white blood cells)

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4
Q

What disease can Myelodysplastic Syndrome with an increase in bone marrow blasts lead to?

A

Acute Myeloid Leukemia

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5
Q

What age group is more commonly affected by Myelodysplastic Syndrome?

A

60+ Years Old

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6
Q

How is Myelodysplastic Syndrome typically discovered in a patient?

A

Incidental finding of abnormal blood counts

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7
Q

When Myelodysplastic Syndrome develops symptoms, it typically presents with fatigue, infection, and bleeding. What is the cause of these symptoms?

A

Cytopenia

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8
Q

What are the physical exam findings of Myelodysplastic Syndrome?

A

Splenomegaly
Pallor
Bleeding
Infection

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9
Q

What would a complete blood count (CBC) show in a patient with Myelodysplastic Syndrome (MDS)

A

Anemia with Normal or Increased MCV

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10
Q

What would a Peripheral Blood Smear show in a patient with Myelodysplastic Syndrome (MDS)

A

Macro-ovalocytes

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11
Q

What would a Bone marrow aspiration or biopsy show in a patient with Myelodysplastic Syndrome (MDS)

A

Hypercellular
Myeloid series often left shifted, with variable increases in blasts

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12
Q

What would a Prussian Blue Stain show in a patient with Myelodysplastic Syndrome?

A

Ringed Sideroblasts

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13
Q

What is the characteristic laboratory abnormality seen in patients with Myelodysplastic Syndrome?

A

Dwarf Megakaryocytes with Unilobed Nucleus

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14
Q

What abnormalities define Myelodysplastic Syndrome?

A

Genetic Abnormalities

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15
Q

What chromosomes have frequent cytogenic abnormalities with Myelodysplastic Syndrome?

A

5 and 7

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16
Q

Myelodysplasia is separated from Acute Myeloid Leukemia by what?

A

Less than 20% blasts

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17
Q

What is the course and prognosis of Myelodysplastic Syndrome?

A

Ultimately fatal disease
Death due to infections or bleeding

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18
Q

What is the only curative therapy for Myelodysplastic Syndrome?

A

Allogenic Transplantation

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19
Q

Leukemias that arise from the lineage that gives rise to granulocytes, erythrocytes, and platelets.

A

Myelgoenous
(AML and CML)

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20
Q

Leukemias that involve the lymphocytic lineage that gives rise to T and B lymphocytes.

A

Lymphocytic
(ALL and CLL)

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21
Q

In chronic Leukemia there a lots of what that are circulating in the blood?

A

Partially developed White Blood Cells (WBC)

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22
Q

Myeloproliferative disorder characterized by the overproduction of myeloid cells.

A

Chronic Myeloid Leukemia (CML)

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23
Q

What is a key feature seen in Chronic Myeloid Leukemia?

A

Philadelphia Chromosomes
(Fusion Gene)

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24
Q

What two chromosomes experience a translocation to form a Philadelphia Chromosome?

A

9 and 22

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25
Q

What phase is most commonly seen in patients with Myeloid Leukemias?

A

Chronic Phase (90%)

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26
Q

What can Chronic Myeloid Leukemia progress to if left untreated?

A

Acute Blast Phase

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27
Q

What patient population is more likely to develop Chronic Myeloid Leukemia?

A

Middle Age
(Median is 55 Years Old)

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28
Q

What can you expect a peripheral blood smear of a patient with Chronic Myeloid Leukemia to show?

A

Myeloid series left shifted with mostly mature cells
(Blasts less than 5%)

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29
Q

What is essential to ensure sufficient material for a complete karyotype and morphologic evaluation to confirm the phase of disease of Chronic Myeloid Leukemia?

A

Bone Marrow Biopsy

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30
Q

When is the Blast Phase of Chronic Myeloid Leukemia diagnosed?

A

Blasts of 20% or more

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31
Q

Priapism, respiratory distress, visual blurring, and altered mental status are all seen in doing what event in patients with Myeloid Leukemia?

A

Extreme Hyperleukocytosis

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32
Q

How is Extreme Hyperleukocytosis treated?

A

Emergent Leukapheresis with Myelosuppressive Therapy

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33
Q

Phase of Chronic Myeloid Leukemia where the goal of therapy is to normalize the hematology abnormalities and suppress the malignant bcr/abl-expressing clone.

A

Chronic-phase Chronic Myeloid Leukemia

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34
Q

What is the treatment of choice for Chronic-phase Chronic Myeloid Leukemia?

A

Tyrosine Kinase Inhibitor
(Imatinib)

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35
Q

How is the Advanced Stage Disease of Chronic Myeloid Leukemia treated?

A

Tyrosine Kinase Inhibitor or Myelosuppressive Therapy
(Consider Stem Cell Transplant)

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36
Q

Chronic malignancy of B lymphocytes.

A

Chronic Lymphocytic Leukemia

37
Q

How does Chronic Lymphocytic Leukemia present?

A

Immunosuppression
Bone Marrow Failure
Organ Infiltration with Lymphocytes

38
Q

What age population is most commonly affected by Chronic Lymphocytic Leukemia?

A

Older Patients
Median Age = 70
(90% are 50+ Years Old)

39
Q

How is Chronic Lymphocytic Leukemia typically discovered?

A

Incidentally

40
Q

What is the hallmark sign of Chronic Lymphocytic Leukemia?

A

Isolated Lymphocytosis

41
Q

Would be seen in the bone marrow of a patient with Chronic Lymphocytic Leukemia?

A

Small Lymphocytes
(Diagnosis made from phenotype and genetic markers)

42
Q

What types of cells are seen in Chronic Lymphocytic Leukemia?

A

Smudge Cells

43
Q

What are Smudge Cells?

A

Cell remnants from fragile cells

44
Q

What is the Standard of Care for Early Indolent Chronic Lymphocytic Leukemia?

A

Observation

45
Q

What are some indications for treatment in a patient with Chronic Lymphocytic Leukemia?

A

Progressive Fatigue
Symptomatic Lymphadenopathy
Anemia
Thrombocytopenia

46
Q

What is the initial treatment for Chronic Lymphocytic Leukemia?

A

Target Biologic Therapy
(BTK inhibitor)

47
Q

What genetic condition is associated with Acute Leukemia?

A

Down Syndrome

48
Q

What is the most common Acute Leukemia of adults?

A

Acute Myeloid Leukemia

49
Q

What is the most common childhood leukemia?

A

Acute Lymphocyte Leukemia

50
Q

What are the two most common findings of Acute Leukemias?

A

Bleeding (thrombocytopenia)
Infection (neutropenia)

51
Q

What is the combination hallmark of Acute Leukemia?

A

Pancytopenia + Circulating Blasts

52
Q

What bone marrows finding would you expect in a patient with an Acute Leukemia?

A

Hypercellular
Blasts > 20%

53
Q

What is a specific characteristic of Acute Myeloid Leukemia?

A

Auer Rod
(eosinophilic need-like inclusion in the cytoplasm)

54
Q

What is the treatment for a patient with Acute Myeloid Leukemia when you have a curative intent?

A

Cytarabine + Daunorubicin or Idarubicin

55
Q

What is the treatment for a patient with Acute Lymphoblastic Leukemia?

A

Daunorubicin
Asparaginase
Vincristine
Prednisone

56
Q

If a patient with Acute Lymphoblastic Leukemia has Philadelphia chromosomes, what should be added to their therapy?

A

Tyrosine Kinase Inhibitor

57
Q

What is the remission rate for a patient with Acute Lymphoblastic Leukemia?

58
Q

Rare malignancy of hematopoietic stem cells differentiated as mature B lymphocytes with hair cytoplasmic projections.

A

Hairy Cell Leukemia

59
Q

What is significant about the bone marrow in Hair Cell Leukemia?

A

Inaspirable
(Dry tap)

60
Q

Proliferation of B or T cells that spread non-contiguously. Usually presenting as enlarged lymph nodes.

A

Non-Hodgkin’s Lymphoma

61
Q

What is the pathogenesis of Non-Hodgkins Lymphoma?

A

Oncogene Overexpression
(due to chromosomal translocations)

62
Q

What percent of Non-Hodgkins Lymphomas affect B-Cells

63
Q

What percent of Non-Hodgkins Lymphomas affect T-Cells or NK-cells in origin?

64
Q

What are some signs and symptoms that are seen in a patient with Non-Hodgkin Lymphomas?

A

Burkitt Lymphoma: abdominal pain or fullness
Extra-nodal sites of disease

65
Q

How is Non-Hodgkin Lymphoma diagnosed?

A

Lymph Node Tissue Biopsy

66
Q

What are the most common indolent lymphomas seen in Non-Hodgkins Lymphomas?

A

Follicular
Marginal Zone
Small Lymphocytic

67
Q

What is the treatment for Diffuse large B-Cell Lymphoma (Non-Hodgkins)

A

R-CHOP
(six cycles of immunochemotherapy)

68
Q

A cancer of the germinal center B cell cells.

A

Hodgkin Lymphoma

69
Q

Hodgkin Lymphoma is characterized by what on a lymph node biopsy?

A

Reed-Sternberg Cells

70
Q

When are people most likely develop Hodgkin Lymphoma?

A

20s
50+ Years Old

71
Q

What is a common presentation of Hodgkin Lymphoma?

A

Painless mass in the neck

72
Q

Subtype of Hodgkin Lymphoma that consists of nodular sclerosis, mixed cellularity of lymphocyte rich and lymphocyte depleted.

A

Classic Hodgkin
(Most Common)

73
Q

Subtype of Hodgkin Lymphoma that consists of a predominant nodular lymphocyte.

A

Non-classic Hodgkin

74
Q

What is required for the diagnosis of Hodgkin Lymphoma?

A

Lymph Node Biopsy

75
Q

What is the mainstay of treatment for a patient with Hodgkin Lymphoma?

A

Chemotherapy

76
Q

What is the treatment of choice for a Low-risk patient with Hodgkin Lymphoma?

A

ABVD
(short course chemotherapy with nodal radiotherapy)

77
Q

What is the treatment of choice for a High-risk patient with Hodgkin Lymphoma?

A

ABVD
(full course of chemotherapy for six cycles)

78
Q

Characterized by the infiltration of the bone marrow, bone destruction, and paraprotein production.

A

Plasma Cell Myeloma
(Multiple Myeloma)

79
Q

In Plasma Cell Myeloma, malignant cells overproduce non-functional antibodies, resulting in immunodeficiency and elevated serum and urine protein. What is this known as?

A

Bence Jones proteinuria

80
Q

How is Plasma Cell Myeloma diagnosed?

A

Serum Protein Electrophoresis
Free Light Chains
Bone Marrow Biopsy

81
Q

Patient’s with Plasma Cell Myeloma are prone to what?

A

Infection via encapsulated organsims
- strep pneumo
- h. flu
VACCINATE

82
Q

What is the median age of diagnosis of Plasma Cell Myeloma?

A

65 Years Old

83
Q

What are the most common presenting complaints with Plasma Cell Myeloma?

A

Bone Pain
Anemia
Kidney Disease
Infection

84
Q

Where is bone pain most commonly located at in patients with Plasma Cell Myeloma?

A

Back (vertebrae)
Hips (femoral neck)
Ribs

85
Q

What is an odd symptom that patients with Plasma Cell Myeloma may present with?

A

Oliguria
(decreased urination)

86
Q

Anemia is nearly universal in patients with Plasma Cell Myeloma. While red blood cell morphology is normal, what is common and may be marked?

A

Rouleaux Formation

87
Q

Serum or Urine protein electrophoresis (PEP) or immunofixation electrophoresis (IFE) will show what hallmark finding in patients with Plasma Cell Myeloma?

A

Paraprotein
(M-protein)

88
Q

What would you expect to see on a bone radiograph of a patient with Plasma Cell Myeloma?

A

Lytic Leasions in Axial Skeleton

89
Q

In the evaluation of patients with known or suspected Plasma Cell Myeloma, what is the preferred method to detect bone disease?