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Clinical Medicine I > Heme malignancies > Flashcards

Heme malignancies Flashcards

1
Q

What is characterized by asymptomatic and incidental finding of abnormal blood counts, with occasional cytopenia (fatigue, infection, bleeding, fever, weight loss, general debility)?

A

myelodysplastic syndrome

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2
Q

What makes someone at risk for myelodysplastic syndrome?

A

prior exposure to cytotoxic chemo and radiation, >60

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3
Q

What is myelodysplastic syndrome?

A

type of leukemia that is a precursor to acute leukemia –> buildup of IMMATURE cells w a “constellation of cytopenias w/ hypercellular marrow, morphologic dysplasia”

increase in bone marrow blasts (more aggressive)

can have presence of defining genetic alterations!

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4
Q

What on a PE would signify myelodysplastic syndrome?

A

splenomegaly, pallor, bleeding, infection

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5
Q

What on a CBC would signify myelodysplastic syndrome?

A

anemia w/ normal or increased MCV, left shift and WBC/platelets normal or reduced

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6
Q

What on a peripheral blood smear would indicate myelodysplastic syndrome?

A

macro-ovalocytes, neutrophils w/ morphologic abnormalities (# of granules, Pelger-Huet abnormality), small # of promyelocytes or blasts, platelets

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7
Q

What’s key about the blast percentage in myelodysplastic syndrome?

A

<20% blasts, if more, then think AML

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8
Q

What on a bone marrow biopsy indicates myelodysplastic syndrome?

A

hypercellular (LOTS of cells), erythroid hyperplasia, abnormal erythropoiesis

Prussian ringed blue stain = ringed sideroblasts

Myeloid series often left shifted w/ increases in blasts, dwarf megakaryocytes w/ unilobed nucleus

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9
Q

How can you craft a diagnosis for myelodysplastic syndrome?

A

with genetic abnormalities! Generally chromosome 5 & 7

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10
Q

How can you treat myelodysplastic syndrome?

A

low risk: erythropoietic growth factors, w/ chromosome abnormalitiy: lenalidomide, immunosuppressive therapy (anti-thymocyte globulin)

high: hypomethylating agents (azacitidine or decitabine), bone marrow transplant

consider transfusions like RBC for anemia, platelets for bleeding

Deferasirox to eliminate iron buildup, abx to prevent infections

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11
Q

What is indicated by a chronic phase sometimes followed by an acute blast phase involving fatigue, night sweats, low-grade fevers, abdominal fullness w/ splenomegaly and with accelerated disease involving fever, bone pain, and splenomegaly?

A

chronic myeloid leukemia

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12
Q

In who is chronic myeloid leukemia more likely?

A

middle age

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13
Q

What causes chronic myeloid leukemia?

A

overproduction of myeloid cells – Philadelphia chromosome of a fusion gene bcr/abl that induces leukemia!

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14
Q

What on a PE could indicate chronic myeloid leukemia?

A

splenomegaly + sternal tenderness

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15
Q

What on a CBC could indicate chronic myeloid leukemia?

A

elevated WBC, left shift, <5% blasts, basophilia and eosinophilia, with normalish platelets (Hct, Hg, RBCs)

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16
Q

What type of test is essential for chronic myeloid leukemia?

A

bone marrow biopsy

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17
Q

What on a bone marrow biopsy could indicate chronic myeloid leukemia?

A

karotype and hypercellular w/ left-shifted myelopoiesis

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18
Q

What’s the hallmark of chronic myeloid leukemia?

A

bcr/abl gene with Philadelphia chromosome, detected by PCR

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19
Q

With chronic myeloid leukemia progression to blast phase, what should indicate that?

A

> 20% of blast M cells and progressive anemia (more pronounced) + thrombocytopenia

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20
Q

How should you treat chronic myeloid leukemia?

A

extreme hyperleukocytosis w/ priapism (resp distress, visual blurring, AMS) - emergent leukapheresis w/ myelosuppressive therapy

chronic phase CML: tyrosine kinase inhibs (imatinib)

advanced stage (blast crisis): tyrosine kinase inhib alone or combo w/ myelosuppressive therapy

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21
Q

What does fatigue or lymphadenopathy with 50% having hepatomegaly or splenomegaly indicate?

A

chronic lymphocytic leukemia

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22
Q

In who is chronic lymphocytic leukemia more likely?

A

> 50y, m: 70

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23
Q

What causes chronic lymphocytic leukemia?

A

malignancy of B lymphocytes with immunosuppression, bone marrow failure, organ infiltration

24
Q

What can indicate chronic lymphocytic leukemia?

A

isolated lymphocytosis!
WBC elevated, Hct and platelets normal initially

bone marrow = small but mature lymphocytes

smudge = cell remnants from fragile cells, lymph nodes infiltrated

25
Q

What are indications for treatment with chronic lymphocytic leukemia?

A

progressive fatigue, symptomatic lymphadenopathy, anemia, thrombocytopenia

26
Q

How do you treat chronic lymphocytic leukemia if it is indicated?

A

targeted biologic therapy – BTK inhibitor (ibrutinib, acalabrutinib, zanubrutinib)
OR
venetoclax w/ anti-CD20 antibody therapy (obinutuzumab)

for super severe = allogeneic transplantation which is high risk but needed for super severe

27
Q

What is indicated by bleeding (such as gingival, epistaxis, menorrhagia, from thrombocytopenia) and infection (like cellulitis, pneumonia, perirectal infections, fungal, death from neutropenia) that risks rise with <500 neutrophil and can have gum hypertrophy, bone/joint pain, and severe illness with hyperleukocytosis w/ headache, confusion, dyspnea?

A

acute myeloid leukemia

28
Q

In who is acute myeloid leukemia common?

A

MC malignancy of adults ~60y and with down syndrome

29
Q

What is acute myeloid leukemia caused by?

A

malignancy of myeloblasts

30
Q

What in a PE does acute myeloid leukemia indicate?

A

pale, purpura, petechiae, stomatitis, gum hypertrophy, rectal fissures, enlargement of liver, spleen, lymph nodes, bone tenderness of sternum, tibia, femur

31
Q

What are some diagnosis features of acute myeloid leukemia?

A

pancytopenia w/ circulating blasts, and bone marrow = hypercellular and dominated by blasts (>20%), hyperuricemia

32
Q

What is key in differential diagnosis of acute myeloid leukemia?

A

Auer rod (eosinophilic needle-like inclusion in cytoplasm)

33
Q

How can you treat acute myeloid leukemia?

A

curative intent – anthracycline (danurubicin or idarubicin) + cytarabine (alone or in combo w/ other agents)

non curative – (>75y or significant comorbidities) newer treatments

34
Q

How can one differentiate acute lymphoblastic leukemia?

A

similar symptoms to AML but “bulky disease”, some lymphadenopathy, hepatosplenomegaly

35
Q

In who is acute lymphoblastic leukemia common?

A

MC acute leukemia of childhood 3-7y, down syndrome

36
Q

What causes acute lymphoblastic leukemia?

A

malignancy of precursor B or T lymphocytes

37
Q

What differentiates acute lymphoblastic leukemia?

A

mediastinal mass visible on CXR

38
Q

What should you remember about treatment for ALL?

A

young adults have better outcomes when treated like peds protocol, with older patients going straight to allogeneic transplantation

39
Q

How do you treat acute lymphoblastic leukemia?

A

combo chemi including daunrubicin, vincristine, prednisone, asparaginase

if they have the philadelphia chromosome, add a tyrosine kinase inhibitor (dasatinib or ponatinib)!

40
Q

How should you treat acute lymphoblastic leukemia after remission?

A

high dose chemo/additional cycles + stem cell transplantation

41
Q

What does enlarged lymph nodes indicate with “B symptoms” being fever, drenching night sweats, weight loss >10% body weight?

A

non-hodgkin’s lymphoma

42
Q

What causes non-hodgkin’s lymphoma?

A

proliferation of B (most) or T cells, spreading non-linear, skipping around to extranodal sites

43
Q

What are some notable things on a non-hodgkin’s lymphoma PE?

A

lymphadenopathy isolated or diffuse, extranodal sites of disease may be found (skin, GI tract, liver, bone marrow)

Burkitt=abdominal pain/fullness

44
Q

How can you help diagnose non-hodgkin’s lymphoma?

A

tissue biopsy for accuracy and classification

high grade –> CSF, serum LD - useful prognostic

45
Q

How can you treat indolent non-hodgkin’s lymphoma?

A

local irradiation w/ cure intent
disseminated disease - not curable, only offered when symptoms develop or tumor bulk

46
Q

How do you treat aggressive non-hodgkin’s lymphoma?

A

R-CHOP

47
Q

What is characterized by a painless mass, commonly in the neck, w/fever, weight loss, drenching night sweats, general pruritus, when drinking, can become painful?

A

hodgkin’s lymphoma

48
Q

What puts someone at risk for hodgkin’s lymphoma?

A

EBV virus, 20s and >50

49
Q

What causes hodgkin’s lymphoma?

A

unknown but spreads contiguously, cancer of germinal center B cells

50
Q

How do you diagnose hodgkin’s lymphoma?

A

lymph node biopsy required for diagnosis – Reed-Sternberg cells are key!

51
Q

How can you treat hodgkin’s lymphoma?

A

chemotherapy with dosing based on risk, with either low risk or high risk, ABVD

52
Q

What is characterized by CRAB - high levels of calcium, decreased renal function, low amount of RBCs, bone damage, and can have hyperviscosity syndrome?

A

plasma cell myeloma/multiple myeloma

53
Q

What causes plasma cell myeloma/multiple myeloma?

A

proliferation of plasma cells in bone which makes them prone to infection, so vaccinate!

54
Q

What on the PE is key for plasma cell myeloma/multiple myeloma?

A

pallor, bone tenderness, soft tissue masses, neurologic signs w/ neuropathy or spinal cord compression, fever w/ infection, oliguria

55
Q

What’s key in labs for plasma cell myeloma/multiple myeloma?

A

Bence Jones proteinuria, anemia (rouleaux formation), lytic lesions in radiograph w/ diagnosis involving analysis of serum protein electrophoresis and free light chains + bone marrow biopsy

56
Q

How can you treat plasma cell myeloma/multiple myeloma?

A

chemo w/ immunomodulatory agent

Clinical Medicine I (33 decks)

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