Hematologic Malignancies Flashcards
Disease that is characterized by a constellation of cytopenias, hypercelluar marrow, morphologic dysplasia, and genetic abnormalities.
Myelodysplastic Syndrome
What are the general causes of Myelodysplastic Syndrome (MDS)
Usually Idiopathic
Cytotoxic Chemotherapy
Radiation
What is the key distinction of Myelodysplastic Syndrome (MDS)
Increase in bone marrow blasts
(immature white blood cells)
What disease can Myelodysplastic Syndrome with an increase in bone marrow blasts lead to?
Acute Myeloid Leukemia
What age group is more commonly affected by Myelodysplastic Syndrome?
60+ Years Old
How is Myelodysplastic Syndrome typically discovered in a patient?
Incidental finding of abnormal blood counts
When Myelodysplastic Syndrome develops symptoms, it typically presents with fatigue, infection, and bleeding. What is the cause of these symptoms?
Cytopenia
What are the physical exam findings of Myelodysplastic Syndrome?
Splenomegaly
Pallor
Bleeding
Infection
What would a complete blood count (CBC) show in a patient with Myelodysplastic Syndrome (MDS)
Anemia with Normal or Increased MCV
What would a Peripheral Blood Smear show in a patient with Myelodysplastic Syndrome (MDS)
Macro-ovalocytes
What would a Bone marrow aspiration or biopsy show in a patient with Myelodysplastic Syndrome (MDS)
Hypercellular
Myeloid series often left shifted, with variable increases in blasts
What would a Prussian Blue Stain show in a patient with Myelodysplastic Syndrome?
Ringed Sideroblasts
What is the characteristic laboratory abnormality seen in patients with Myelodysplastic Syndrome?
Dwarf Megakaryocytes with Unilobed Nucleus
What abnormalities define Myelodysplastic Syndrome?
Genetic Abnormalities
What chromosomes have frequent cytogenic abnormalities with Myelodysplastic Syndrome?
5 and 7
Myelodysplasia is separated from Acute Myeloid Leukemia by what?
Less than 20% blasts
What is the course and prognosis of Myelodysplastic Syndrome?
Ultimately fatal disease
Death due to infections or bleeding
What is the only curative therapy for Myelodysplastic Syndrome?
Allogenic Transplantation
Leukemias that arise from the lineage that gives rise to granulocytes, erythrocytes, and platelets.
Myelgoenous
(AML and CML)
Leukemias that involve the lymphocytic lineage that gives rise to T and B lymphocytes.
Lymphocytic
(ALL and CLL)
In chronic Leukemia there a lots of what that are circulating in the blood?
Partially developed White Blood Cells (WBC)
Myeloproliferative disorder characterized by the overproduction of myeloid cells.
Chronic Myeloid Leukemia (CML)
What is a key feature seen in Chronic Myeloid Leukemia?
Philadelphia Chromosomes
(Fusion Gene)
What two chromosomes experience a translocation to form a Philadelphia Chromosome?
9 and 22
What phase is most commonly seen in patients with Myeloid Leukemias?
Chronic Phase (90%)
What can Chronic Myeloid Leukemia progress to if left untreated?
Acute Blast Phase
What patient population is more likely to develop Chronic Myeloid Leukemia?
Middle Age
(Median is 55 Years Old)
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%)
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
When is the Blast Phase of Chronic Myeloid Leukemia diagnosed?
Blasts of 20% or more
Priapism, respiratory distress, visual blurring, and altered mental status are all seen in doing what event in patients with Myeloid Leukemia?
Extreme Hyperleukocytosis
How is Extreme Hyperleukocytosis treated?
Emergent Leukapheresis with Myelosuppressive Therapy
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
What is the treatment of choice for Chronic-phase Chronic Myeloid Leukemia?
Tyrosine Kinase Inhibitor
(Imatinib)
How is the Advanced Stage Disease of Chronic Myeloid Leukemia treated?
Tyrosine Kinase Inhibitor or Myelosuppressive Therapy
(Consider Stem Cell Transplant)
Chronic malignancy of B lymphocytes.
Chronic Lymphocytic Leukemia
How does Chronic Lymphocytic Leukemia present?
Immunosuppression
Bone Marrow Failure
Organ Infiltration with Lymphocytes
What age population is most commonly affected by Chronic Lymphocytic Leukemia?
Older Patients
Median Age = 70
(90% are 50+ Years Old)
How is Chronic Lymphocytic Leukemia typically discovered?
Incidentally
What is the hallmark sign of Chronic Lymphocytic Leukemia?
Isolated Lymphocytosis
Would be seen in the bone marrow of a patient with Chronic Lymphocytic Leukemia?
Small Lymphocytes
(Diagnosis made from phenotype and genetic markers)
What types of cells are seen in Chronic Lymphocytic Leukemia?
Smudge Cells
What are Smudge Cells?
Cell remnants from fragile cells
What is the Standard of Care for Early Indolent Chronic Lymphocytic Leukemia?
Observation
What are some indications for treatment in a patient with Chronic Lymphocytic Leukemia?
Progressive Fatigue
Symptomatic Lymphadenopathy
Anemia
Thrombocytopenia
What is the initial treatment for Chronic Lymphocytic Leukemia?
Target Biologic Therapy
(BTK inhibitor)
What genetic condition is associated with Acute Leukemia?
Down Syndrome
What is the most common Acute Leukemia of adults?
Acute Myeloid Leukemia
What is the most common childhood leukemia?
Acute Lymphocyte Leukemia
What are the two most common findings of Acute Leukemias?
Bleeding (thrombocytopenia)
Infection (neutropenia)
What is the combination hallmark of Acute Leukemia?
Pancytopenia + Circulating Blasts
What bone marrows finding would you expect in a patient with an Acute Leukemia?
Hypercellular
Blasts > 20%
What is a specific characteristic of Acute Myeloid Leukemia?
Auer Rod
(eosinophilic need-like inclusion in the cytoplasm)
What is the treatment for a patient with Acute Myeloid Leukemia when you have a curative intent?
Cytarabine + Daunorubicin or Idarubicin
What is the treatment for a patient with Acute Lymphoblastic Leukemia?
Daunorubicin
Asparaginase
Vincristine
Prednisone
If a patient with Acute Lymphoblastic Leukemia has Philadelphia chromosomes, what should be added to their therapy?
Tyrosine Kinase Inhibitor
What is the remission rate for a patient with Acute Lymphoblastic Leukemia?
90%
Rare malignancy of hematopoietic stem cells differentiated as mature B lymphocytes with hair cytoplasmic projections.
Hairy Cell Leukemia
What is significant about the bone marrow in Hair Cell Leukemia?
Inaspirable
(Dry tap)
Proliferation of B or T cells that spread non-contiguously. Usually presenting as enlarged lymph nodes.
Non-Hodgkin’s Lymphoma
What is the pathogenesis of Non-Hodgkins Lymphoma?
Oncogene Overexpression
(due to chromosomal translocations)
What percent of Non-Hodgkins Lymphomas affect B-Cells
85%
What percent of Non-Hodgkins Lymphomas affect T-Cells or NK-cells in origin?
15%
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
How is Non-Hodgkin Lymphoma diagnosed?
Lymph Node Tissue Biopsy
What are the most common indolent lymphomas seen in Non-Hodgkins Lymphomas?
Follicular
Marginal Zone
Small Lymphocytic
What is the treatment for Diffuse large B-Cell Lymphoma (Non-Hodgkins)
R-CHOP
(six cycles of immunochemotherapy)
A cancer of the germinal center B cell cells.
Hodgkin Lymphoma
Hodgkin Lymphoma is characterized by what on a lymph node biopsy?
Reed-Sternberg Cells
When are people most likely develop Hodgkin Lymphoma?
20s
50+ Years Old
What is a common presentation of Hodgkin Lymphoma?
Painless mass in the neck
Subtype of Hodgkin Lymphoma that consists of nodular sclerosis, mixed cellularity of lymphocyte rich and lymphocyte depleted.
Classic Hodgkin
(Most Common)
Subtype of Hodgkin Lymphoma that consists of a predominant nodular lymphocyte.
Non-classic Hodgkin
What is required for the diagnosis of Hodgkin Lymphoma?
Lymph Node Biopsy
What is the mainstay of treatment for a patient with Hodgkin Lymphoma?
Chemotherapy
What is the treatment of choice for a Low-risk patient with Hodgkin Lymphoma?
ABVD
(short course chemotherapy with nodal radiotherapy)
What is the treatment of choice for a High-risk patient with Hodgkin Lymphoma?
ABVD
(full course of chemotherapy for six cycles)
Characterized by the infiltration of the bone marrow, bone destruction, and paraprotein production.
Plasma Cell Myeloma
(Multiple Myeloma)
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
How is Plasma Cell Myeloma diagnosed?
Serum Protein Electrophoresis
Free Light Chains
Bone Marrow Biopsy
Patient’s with Plasma Cell Myeloma are prone to what?
Infection via encapsulated organsims
- strep pneumo
- h. flu
VACCINATE
What is the median age of diagnosis of Plasma Cell Myeloma?
65 Years Old
What are the most common presenting complaints with Plasma Cell Myeloma?
Bone Pain
Anemia
Kidney Disease
Infection
Where is bone pain most commonly located at in patients with Plasma Cell Myeloma?
Back (vertebrae)
Hips (femoral neck)
Ribs
What is an odd symptom that patients with Plasma Cell Myeloma may present with?
Oliguria
(decreased urination)
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
Serum or Urine protein electrophoresis (PEP) or immunofixation electrophoresis (IFE) will show what hallmark finding in patients with Plasma Cell Myeloma?
Paraprotein
(M-protein)
What would you expect to see on a bone radiograph of a patient with Plasma Cell Myeloma?
Lytic Leasions in Axial Skeleton
In the evaluation of patients with known or suspected Plasma Cell Myeloma, what is the preferred method to detect bone disease?
MRI
PET
CT