Heme #6: AML, ALL, CML, CLL, MM Flashcards

1
Q

What do myeloid cells give rise to?

What do lymphoid cells give rise to?

A

Myeloid: RBCs

Lymphoid: Lymphocytes (WBCs) and natural killer cells

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2
Q

Acute Lymphocytic Leukemia
-MC childhood malignancy (peak is ages 2-5). Other risk factors are…
-Pathophysiology of this condition
-Symptoms as a result of

A

-RF: Boys, Down Syndrome, Ages 2-5
-Overpopulation of immature WBC’s (blasts) overtake normal hematopoeisis, resulting in pancytopenia
-Symptoms: fever and infections (leukopenia), bleeding from thrombocytopenia, petechiae, purpura, anemia, pallor, fatigue.
–CNS symptoms: stiff neck, visual changes, METs to CNS and testes MC.
–Hepatosplenomegaly, abdominal pain, LAD

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3
Q

What is the MC physical exam finding of ALL?

What should you remember to remember this condition?

A

-Hepatomegaly or splenomegaly

ALL my children. MC childhood malignancy.

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4
Q

Diagnostics for ALL
-CBC + peripheral smear
-Bone marrow aspiration
-What is the most accurate test to determine different types of leukemia?

A

-CBC: WBC 5,000 - 100,000, thrombocytopenia
-Bone marrow aspiration: hypercellular with > 20% blasts (DEFINITIVE)
-Flow cytometry test is the best way to distinguish subtypes of leukemia

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5
Q

ALL is/isn’t highly response to combination chemotherapy?

If the patient has CNS disease, what medication can be used as treatment?

A

It is highly responsive to combination chemotherapy!

-Intrathecal Methotrexate

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6
Q

What is tumor lysis syndrome?
-Remember, it is related to the leukemias.

A

-Emergency that occurs after treatment due to rapid tumor cell lysis after chemotherapy, resulting in potassium, phosphate, and nucleic acids into circulation

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7
Q

-Symptoms of tumor lysis syndrome

-Lab findings with tumor lysis syndrome

A

-Symptoms: muscle cramps, tetany, nausea, vomiting, lethargy, HF, kidney injury

-Hypocalcemia
-Hyperuricemia, hyperphosphatemia, hyperkalemia, and acute kidney injury

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8
Q

How do you treat tumor lysis syndrome?

How can you prevent tumor lysis syndrome with medication?

A

-Treat electrolyte abnormalities, IVF

-Rasburicase or Allopurinol (catalyzes uric acid to a stable compound)

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9
Q

Chronic Lymphocytic Leukemia (CLL) is
-The MC form of leukemia in adults
-Mature B cell clonal malignancy
-Risk Factors?

A

-Increasing age (70 years is median age)
-Male gender

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10
Q

CLL symptoms

A

-Usually asymptomatic
-Pancytopenia: anemia symptoms (pallor, fatigue MC, dyspnea), neutropenia (increased infections), and thrombocytopenia (mucocutaneous bleeding)

-Lymphadenopathy MC exam finding, Splenomegaly, skin lesions (leukemia cutis)

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11
Q

What is seen on CBC with peripheral smear for CLL?

What other unique lab finding?

A

-Absolute lymphocytosis: small, well-differentiated normal-appearing lymphocytes with scattered “smudge cells”
–Smudge cells: fragile B cells become crushed by cover slip during slide preparation

-Hypogammaglobulinemia: increased incidence of autoimmune hemolytic anemia.

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12
Q

Management for CLL

A

-Chemotherapy

-Allogeneic stem cell transplant curative

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13
Q

Acute Myeloid Leukemia (AML)
-MC acute leukemia in adults
-Pathophysiology
-MC subtype

A

-Patho: accumulation of leukemia blasts (immature WBCs) in the bone marrow –> pancytopenia
-Acute promyelotic leukemia (APL or M3): associated with DIC, presence of Auer rods, and myeloperoxidase positivity

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14
Q

Symptoms of AML

A

-Pancytopenia: anemia (general fatigue MC initial symptom), thrombocytopenia (bleeding), neutropenia (infections)

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15
Q

What is seen on
-CBC with peripheral smear:
-Bone marrow biopsy (GOLD STANDARD):
-Immunophenotyping:

For AML

A

-CBC (best initial test): normocytic normochromic anemia. Thrombocytopenia. Circulating myeloblasts.

-Bone marrow biopsy (GOLD): >20% myeloblasts. Auer Rods (pink/red rods in cytoplasm) with APL.

-Immunophenotyping: Myeloperoxidase positivity with APL.

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16
Q

Management for AML

A

-Combination chemotherapy
-Al-trans-retinoic acid added for M3**

17
Q

What is a leukostasis reaction (associated with AML and CML)?

Explain the pathophysiology of this.

What are symptoms/seen on diagnostics?

A

-Leukostasis leads to white cell plugs in microvasculature, impeding blood flow, causing local hypoxemia

-Hyperleukocytosis (WBC > 100,000) + symptoms due to tissue hypoxia (lung, CNS, heart, etc.)

18
Q

Management for a leukostasis reaction

A

-Leukapheresis (has rapid improvement): removes WBCs from the blood

19
Q

Chronic Myelogenous Leukemia (CML)
-What is it?
-Pathophysiology (two genes remember)
-Symptoms

A

-Uncontrolled production of mature and maturing granulocytes (predominantly neutrophils, but also basophils and eosinophils)

-Fusion of BCR (on chromosome 22) and ABL1 (on chromosome 9), resulting in BCR-ABL1 fusion gene. Translocation of 9 and 22 = Philadelphia chromosome.

-Symptoms: fatigue, night sweats, splenomegaly (MC), pruritus after hot showers/baths

20
Q

Diagnostics for CML
-CBC with peripheral smear:
-Leukocyte alkaline phosphatase score:
-Genetic testing for…

A

-CBC: leukocytosis with granulocytic cells (neutrophilia, basophilia, eosinophilia)

-LAP score decreased. LAP only found in functioning WBCs not leukemic cells.

Genetic testing for Philadelphia chromosome.

21
Q

Management for CML

A

-Tyrosine kinase inhibitors (Imatinib, Nilotinib, Dasatinib)

-Hematopoietic stem cell transplant most effective cure.

22
Q

Multiple Myeloma (Plasmacytoma)
-What is it
-MC primary bone malignancy in adults
-Pathophysiology

A

Cancer due to proliferation of single clone of plasma cells, leading to increased production of ineffective monoclonal antibodies (IgG, IgA, and IgM)

Plasma cells accumulate in the bone marrow, interrupting normal cell production –> kidney injury

23
Q

Symptoms of Multiple Myeloma
-Think BREAK your bones

A

B: Bone pain (MC): Vertebral MC, ribs. Pathologic fractures, osteolytic lesions, radiculopathy
R: Recurrent infections (ineffective IgG)
E: Elevated calcium (bone destruction)
A: Anemia (fatigue, pallor, weight loss)
K: kidney injury (increased BUN and Cr)

24
Q

Diagnostics for Multiple Myeloma
-Serum protein electrophoresis
-Urine protein electrophoresis
-CBC
-Skull radiographs
-Bone marrow aspiration (definitive)

A

-Protein: monoclonal protein spike: IgG MC
-Urine: Bence-Jones protein (kappa or lambda chains)
-CBC: Rouleaux formation (RBCs with stack of coins appearance)
-Skull xrays: punched out, lytic lesions.
-Bone marrow: plasmacytosis > 10%

25
Q

Treatment for Multiple Myeloma

A

-Autologous stem cell transplant most effective
–Chemotherapy is a temporary fix for symptoms