Hematologic Malignancies Flashcards

(89 cards)

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
What phase is most commonly seen in patients with Myeloid Leukemias?
Chronic Phase (90%)
26
What can Chronic Myeloid Leukemia progress to if left untreated?
Acute Blast Phase
27
What patient population is more likely to develop Chronic Myeloid Leukemia?
Middle Age (Median is 55 Years Old)
28
What can you expect a peripheral blood smear of a patient with Chronic Myeloid Leukemia to show?
Myeloid series left shifted with mostly mature cells (Blasts less than 5%)
29
What is essential to ensure sufficient material for a complete karyotype and morphologic evaluation to confirm the phase of disease of Chronic Myeloid Leukemia?
Bone Marrow Biopsy
30
When is the Blast Phase of Chronic Myeloid Leukemia diagnosed?
Blasts of 20% or more
31
Priapism, respiratory distress, visual blurring, and altered mental status are all seen in doing what event in patients with Myeloid Leukemia?
Extreme Hyperleukocytosis
32
How is Extreme Hyperleukocytosis treated?
Emergent Leukapheresis with Myelosuppressive Therapy
33
Phase of Chronic Myeloid Leukemia where the goal of therapy is to normalize the hematology abnormalities and suppress the malignant bcr/abl-expressing clone.
Chronic-phase Chronic Myeloid Leukemia
34
What is the treatment of choice for Chronic-phase Chronic Myeloid Leukemia?
Tyrosine Kinase Inhibitor (Imatinib)
35
How is the Advanced Stage Disease of Chronic Myeloid Leukemia treated?
Tyrosine Kinase Inhibitor or Myelosuppressive Therapy (Consider Stem Cell Transplant)
36
Chronic malignancy of B lymphocytes.
Chronic Lymphocytic Leukemia
37
How does Chronic Lymphocytic Leukemia present?
Immunosuppression Bone Marrow Failure Organ Infiltration with Lymphocytes
38
What age population is most commonly affected by Chronic Lymphocytic Leukemia?
Older Patients Median Age = 70 (90% are 50+ Years Old)
39
How is Chronic Lymphocytic Leukemia typically discovered?
Incidentally
40
What is the hallmark sign of Chronic Lymphocytic Leukemia?
Isolated Lymphocytosis
41
Would be seen in the bone marrow of a patient with Chronic Lymphocytic Leukemia?
Small Lymphocytes (Diagnosis made from phenotype and genetic markers)
42
What types of cells are seen in Chronic Lymphocytic Leukemia?
Smudge Cells
43
What are Smudge Cells?
Cell remnants from fragile cells
44
What is the Standard of Care for Early Indolent Chronic Lymphocytic Leukemia?
Observation
45
What are some indications for treatment in a patient with Chronic Lymphocytic Leukemia?
Progressive Fatigue Symptomatic Lymphadenopathy Anemia Thrombocytopenia
46
What is the initial treatment for Chronic Lymphocytic Leukemia?
Target Biologic Therapy (BTK inhibitor)
47
What genetic condition is associated with Acute Leukemia?
Down Syndrome
48
What is the most common Acute Leukemia of adults?
Acute Myeloid Leukemia
49
What is the most common childhood leukemia?
Acute Lymphocyte Leukemia
50
What are the two most common findings of Acute Leukemias?
Bleeding (thrombocytopenia) Infection (neutropenia)
51
What is the combination hallmark of Acute Leukemia?
Pancytopenia + Circulating Blasts
52
What bone marrows finding would you expect in a patient with an Acute Leukemia?
Hypercellular Blasts > 20%
53
What is a specific characteristic of Acute Myeloid Leukemia?
Auer Rod (eosinophilic need-like inclusion in the cytoplasm)
54
What is the treatment for a patient with Acute Myeloid Leukemia when you have a curative intent?
Cytarabine + Daunorubicin or Idarubicin
55
What is the treatment for a patient with Acute Lymphoblastic Leukemia?
Daunorubicin Asparaginase Vincristine Prednisone
56
If a patient with Acute Lymphoblastic Leukemia has Philadelphia chromosomes, what should be added to their therapy?
Tyrosine Kinase Inhibitor
57
What is the remission rate for a patient with Acute Lymphoblastic Leukemia?
90%
58
Rare malignancy of hematopoietic stem cells differentiated as mature B lymphocytes with hair cytoplasmic projections.
Hairy Cell Leukemia
59
What is significant about the bone marrow in Hair Cell Leukemia?
Inaspirable (Dry tap)
60
Proliferation of B or T cells that spread non-contiguously. Usually presenting as enlarged lymph nodes.
Non-Hodgkin's Lymphoma
61
What is the pathogenesis of Non-Hodgkins Lymphoma?
Oncogene Overexpression (due to chromosomal translocations)
62
What percent of Non-Hodgkins Lymphomas affect B-Cells
85%
63
What percent of Non-Hodgkins Lymphomas affect T-Cells or NK-cells in origin?
15%
64
What are some signs and symptoms that are seen in a patient with Non-Hodgkin Lymphomas?
Burkitt Lymphoma: abdominal pain or fullness Extra-nodal sites of disease
65
How is Non-Hodgkin Lymphoma diagnosed?
Lymph Node Tissue Biopsy
66
What are the most common indolent lymphomas seen in Non-Hodgkins Lymphomas?
Follicular Marginal Zone Small Lymphocytic
67
What is the treatment for Diffuse large B-Cell Lymphoma (Non-Hodgkins)
R-CHOP (six cycles of immunochemotherapy)
68
A cancer of the germinal center B cell cells.
Hodgkin Lymphoma
69
Hodgkin Lymphoma is characterized by what on a lymph node biopsy?
Reed-Sternberg Cells
70
When are people most likely develop Hodgkin Lymphoma?
20s 50+ Years Old
71
What is a common presentation of Hodgkin Lymphoma?
Painless mass in the neck
72
Subtype of Hodgkin Lymphoma that consists of nodular sclerosis, mixed cellularity of lymphocyte rich and lymphocyte depleted.
Classic Hodgkin (Most Common)
73
Subtype of Hodgkin Lymphoma that consists of a predominant nodular lymphocyte.
Non-classic Hodgkin
74
What is required for the diagnosis of Hodgkin Lymphoma?
Lymph Node Biopsy
75
What is the mainstay of treatment for a patient with Hodgkin Lymphoma?
Chemotherapy
76
What is the treatment of choice for a Low-risk patient with Hodgkin Lymphoma?
ABVD (short course chemotherapy with nodal radiotherapy)
77
What is the treatment of choice for a High-risk patient with Hodgkin Lymphoma?
ABVD (full course of chemotherapy for six cycles)
78
Characterized by the infiltration of the bone marrow, bone destruction, and paraprotein production.
Plasma Cell Myeloma (Multiple Myeloma)
79
In Plasma Cell Myeloma, malignant cells overproduce non-functional antibodies, resulting in immunodeficiency and elevated serum and urine protein. What is this known as?
Bence Jones proteinuria
80
How is Plasma Cell Myeloma diagnosed?
Serum Protein Electrophoresis Free Light Chains Bone Marrow Biopsy
81
Patient's with Plasma Cell Myeloma are prone to what?
Infection via encapsulated organsims - strep pneumo - h. flu VACCINATE
82
What is the median age of diagnosis of Plasma Cell Myeloma?
65 Years Old
83
What are the most common presenting complaints with Plasma Cell Myeloma?
Bone Pain Anemia Kidney Disease Infection
84
Where is bone pain most commonly located at in patients with Plasma Cell Myeloma?
Back (vertebrae) Hips (femoral neck) Ribs
85
What is an odd symptom that patients with Plasma Cell Myeloma may present with?
Oliguria (decreased urination)
86
Anemia is nearly universal in patients with Plasma Cell Myeloma. While red blood cell morphology is normal, what is common and may be marked?
Rouleaux Formation
87
Serum or Urine protein electrophoresis (PEP) or immunofixation electrophoresis (IFE) will show what hallmark finding in patients with Plasma Cell Myeloma?
Paraprotein (M-protein)
88
What would you expect to see on a bone radiograph of a patient with Plasma Cell Myeloma?
Lytic Leasions in Axial Skeleton
89
In the evaluation of patients with known or suspected Plasma Cell Myeloma, what is the preferred method to detect bone disease?
MRI PET CT