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Malignant Heme Flashcards

1
Q

Meds associated with aplastic anemia

A

Anti thyroid meds, beta-pa tams, sulfonamides, NSAIDs, anticonvulsants, gold

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2
Q

Treatment for Aplastic Anemia

A

Allogenic hematopoietic stem cell transplant

OR

Antithymocyte globulin, cyclosporine, prednisone

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3
Q

What are the causes of pure red cell aplasia?

A 
Parvovirus B19
Thymoma 
Autoimmune disease
Large granular lymphocyte leukemia
Lymphomas
Solid Tumors
Drugs (phenytoin, INH)
Pregnancy
Anti-EPO Ab (for patients on EPO)
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4
Q

Treatment for idiopathic Pure Red Cell Aplasia.

A

Immunosuppression

Ex. Pred, cyclosporine, and cyclophosphamide

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5
Q

What is the most common cytopenia seen in MDS?

A

Macrocytic anemia

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6
Q

What can MDS transition into?

A

AML

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7
Q

What is the only curative option for MDS?

A

Allogenic Hematopoietic Stem Cell Transplant

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8
Q

What are the most common subtypes of MDS?

A

RAEB-1 (blasts 5-9%, unilineage or multilineage dysplasia)

RAEB-2 blasts 10-19%, unilineage or multilineage dysplasia)

All other WHO types have marrow blasts <5%

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9
Q

If someone is at high risk for AML conversion from MDS, what are your treatment options?

A

Hypomethalating agents (azacytidine and decitabine)

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10
Q

What is the significance of a -5q mutation in MDS?

A

You can treat with lenalidomide (reduces transfusion requirements)

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11
Q

What is the CML mutation and what does it do?

A

t(9;22) = Philadelphia chromosome

BRC-ABL fusion gene that codes for an abnormal tyrosine kinase —> dysregulated cell proliferation

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12
Q

Peripheral blood findings in CML

A

Neutrophilia (rule out leukemoid reaction), myelocytes, metamyelocytes

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13
Q

Phases of CML

A 
Chronic Phase (<10% peripheral blasts)
Accelerated Phase
Blast Crisis (2/2 AML)
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14
Q

Treatment of CML

A

TKIs (imatinib, dasatinib, nilotinib) for chronic phase

Allogenic Hematopoietic Stem Cell transplant (accelerated or blast crisis)

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15
Q

Adverse effects of TKIs

A

Fluid retention

QTc prolongation

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16
Q

Mutation seen in PV

A

JAK2 V617F (in 97% of patients)

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17
Q

Hemoglobin level at diagnosis for patients with PV

A

Men >16.5

Women >16

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18
Q

What may be a hint on differential that a patient has PV rather than secondary erythrocytosis?

A

Basophilia

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19
Q

What is the EPO level in PV?

A

Low (high in secondary causes)

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20
Q

What disease processes can cause erythrocytosis via ectopic/excessive EPO?

A

RCC
RAS
HCC
Fibroids

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21
Q

What is the major complication of PV?

A
  • Arterial and venous thrombosis
  • Progression to post-PV Myelofibrosis
  • Transformation to AML
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22
Q

Goal hematocrit level for phlebotomy in PV

A

Less than 45%

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23
Q

Other treatments for PV

A
  • Aspirin
  • hydroxyurea (>60 or h/o thrombosis)
  • ruxolitinib (JAK 1/2 inhibitor) if refractory
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24
Q

Platelet count in ET

A

> 450k

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25
Q

Mutations associated with ET

A

JAK2 (50%)
Calreticulin
MPL Gene

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26
Q

At what platelet count do you see hemorrhage with ET? And why?

A

> 1.5 million (or with high dose ASA)

Via acquired Von Willebrand Disease

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27
Q

When do you treat ET and with what?

A

Low dose ASA: High risk (>60, h/o thrombosis) and low risk with vasomotor symptoms

Hydroxyurea: platelets >1 million or high risk patients

Anagrelide or interferon-alpha: resistant

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28
Q

Which MPN is LEAST likely to progress to myelofibrosis or AML?

A

ET

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29
Q

Which MPN has the worst prognosis?

A

Primary myelofibrosis

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30
Q

What is an odd way PMF can present?

A

GI bleed (complications of portal hypertension)

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31
Q

Treatment for PMF.

A

HLA Matched allogenic Hematopoietic Stem Cell Transplant

If NOT a candidate, can use:
Ruxolitinib (for splenomegy and hypercatabolic symptoms)

Also consider hydroxyurea and splenectomy

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32
Q

Which cell type is increased in PMF that leads to marrow fibrosis?

A

Megakaryocytes (secrete excess fibroblast growth factor)

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33
Q

Causes of Hypereosinophilia

A

C: collagen vascular disease (ex eosinophilia GPA)
H: Helminthic infection
I: idiopathic
N: Neoplasm like lymphoma
A: Allergy (drugs like sulfas and carbamamezpine), atopy, asthma

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34
Q

What eosinophil count is diagnostic of hypereosinophilic syndrome (HES)?

A

> 1500/uL

35
Q

Treatment for idiopathic HES

A

Glucocorticoids

Imatinib if associated platelet-derived growth factor mutation is present

36
Q

Level of clonal abnormal cells present in bone marrow or blood to diagnose acute leukemia

A

20%

37
Q

Most common acute leukemia in adults

A

AML

38
Q

Smear findings in AML

A

Elevated leukocytes with low ANC, thrombocytopenia, anemia, myeloblasts (sometimes)

39
Q

Cure rates for AML

A

35-40%- Adults younger than 60

5-15%- Adults >60

40
Q

What are the unique features of APL?

A

Leukocytes with primary granules (mad coagulopathy)

Chromosomal translocation t(15;17)

41
Q

How to you treat/cure APL?

A

ATRA

42
Q

Name one good and one bad molecular marker for AML

A

Good: NPM1 (50%)
Bad: FLT3-ITD (33%)- consider transplant in first remission

43
Q

AML induction therapy

A

Anthracycline and cytarabine

44
Q

AML response rate to induction

A

Complete response in 60-85% younger than 60

45
Q

AML Consolidation considerations

A

Low risk: conventional chemo

High risk: allogenic Hematopoietic Stem Cell Transplant

46
Q

Geriatric AML treatment

A 
Supportive care/hospice
Single agent chemo (oral hydroxyurea, low dose cytarabine) OR
Hypomethalating Agents (decitabine, azacitidine)
47
Q

What types of WBCs are involved in ALL in adults? How do these two types present?

A

B-Cell (75%)- extramedullary Disease (GI or testicular involvement)
T-Cell - mediastinal mass with wheeze or skin involvement

48
Q

What mutation can be seen in ALL?

A

Philadelphia chromosome (20-30%)

49
Q

How does treatment change in ALL if the Philadelphia chromosome is present?

A

Need additional intensive consolidation (autologous or allogenic hematopoietic stem cell transplant) after treatment with TKI (ex imatinib)

50
Q

Treatment for ALL

A

Induction: Vincristine, Anthracycline, Steroids, L-asparaginase

*also need CNS prophylaxis

Maintenance: oral mercaptopurine and methotrexate (for up to 2 years)

51
Q

Side effects of L-asparaginase

A

Hypofibrinogenemia

Hypertriglyceridemia

52
Q

Incidence of secondary cancer after childhood ALL

A

11% at 30 years (after radiation therapy)

53
Q

EPO hemoglobin target ESRD

A

12g/dL

54
Q

Indications for G-CSF

A

-Prevention of febrile neutropenia in those on:
— high dose chemo for breast cancer
— intensive chemo for diffuse lymphoma in older patients >65

NOT indicated during induction of acute leukemia

55
Q

Indication for autologous stem cell transplant

A

MM

Relapsed Aggressive Lymphoma

56
Q

Who is the typical donor for Allogenic hematopoietic stem cell transplant?

A

HLA matched sibling

57
Q

What percent of adults over 50 have MGUS?

A

3%

58
Q

Initial workup for plasma cell dyscrasia

A

SPEP
Serum immunofixation
Serum free light chains

59
Q

What should be used to look for lyric lesions in MM?

A

Skeletal survey (NOT bone scan)

60
Q

Features of MM

A

C: Hypercalcemia >11 or >1 from the ULN (PTH independent)
R: renal failure with Cr >2 or CrCl <40
A: Anemia (normocytic or macrocytic) Hgb <10 or 2 below LLN, can see roleaux
B: >= 1 Lytic bone lesions

61
Q

Which features must you have to not biopsy a patient who has a monoclonal gammopathy?

A

IgG <1.5 g/dL
Normal serum free light chains
No end organ damage

62
Q

Features of MGUS

A

M Protein <3 g/dL or Urinary M Protein (<500/24h)
*if >1.5 high risk

With

<10% bone marrow clonal plasma cells

63
Q

Difference between smoldering MM and smoldering WM?

A

Both with bone marrow clonal plasma cells (>10%) but

Myeloma is IgA or IgG >= 3 g/dL
WM is IgM >= 3 g/dL

64
Q

When do you treat MM or WM?

A

M protein present with >10% bone marrow clonal plasma cells and disease specific symptoms

65
Q

When do you say someone has multiple myeloma?

A

> = 60% clonal plasma cells in marrow
Involved:Uninvolved FLC ratio >10 (0.01 or less or 100 or greater)
= 1 focal lesion on MRI

66
Q

Signs and symptoms of WM.

A

Systemic: fatigue, b-symptoms, neuropathy, hyperviscosity, mucosal bleeding
Physical exam: adenopathy, HSM
Labs: anemia, thrombocytopenia

67
Q

Lost the three types of MM

A

Non-IgM (1% progression to MM per year)
IgM (1.5% progression to WM)
Light chain (0.3% progression to PCD)

68
Q

What is an abnormal free light chain ratio that increases risk of progression of smoldering myeloma?

A

<0.125 or >8

69
Q

What is the percent conversion of smoldering myeloma to multiple myeloma?

A

10% per year for the first 5 years

70
Q

MM Treatment

A 
Induction Chemo (melphalan)
Autologous Hematopoietic Stem Cell Transplant 
Observation/Maintenance

*all patients need bisphosphonates and pneumonia/flu shots!

71
Q

Lenalidomide and Pomalidide side effect

A

VTE (need thromboprophylaxis)

72
Q

Bortezomib side effect

A 
Peripheral Neuropathy
Herpes reactivation (need acyclovir)
73
Q

Most common type of amyloidosis

A

AL Amyloid

74
Q

Diseases associated with AL Amyloid

A

PCDs

Waldenström macroglobulinemia (rare)

75
Q

Major organ system impacts of AL amyloid

A

Cardiac- need cardiac MRI with late gadolinium enhancement)
Kidney- nephrotic range proteinuria
GI- GI bleed, delayed emptying, SIBO
Liver- cholestatic pattern, HSM, portal HTN
Neuro- distal sensorimotor polyneuropathy and autonomic neuropathy
Heme- factor x deficiency with long PT and PTT causing periorbital purpura
MSK: CTS, macroglossia

76
Q

Treatment of AL Amyloid

A

Autologous Stem Cell Transplant

Prognosis is dependent on cardiac involvement

77
Q

What makes you ineligible for Autologous Stem Cell Transplant in AL amyloid?

What are you treated with instead?

A

Age >70
Advanced cardiac disease
Stage IV CKD
Large effusions

Get treated with Melphalan or Bortezomib based chemo

78
Q

Diseases associated with AA amyloid

A

RA
IND
Familial Mediterranean Fever
Chronic disease

79
Q

What amyloid protein is deposited in Hereditary Amyloidosis

A

Mutated TTR or fibrinogen alpha chain

80
Q

What type of amyloid protein is deposited in dialysis related amyloidosis?

A

Beta-2 microglobulin

81
Q

Major complication of WM and how do you treat?

A

Hyperviscosity Syndrome

Treat with plasmapheresis then targeted therapy with
rituximab (+- chemo)
Steroids,

*new TKI approved= ibrutinib

82
Q

What is cryoglobulinemia?

A

Clonal or polyclonal Ig’s that precipitate at temp <37C and dissolve when rewarmed

83
Q

Features of Type I Cryoglobulinemia

A 
Monoclonal Immunoglobulin (IgM)
Associated with Plasma cell dyscrasias
Usually asymptomatic (May have ulcers, Raynaud phenomenon, digital ischemia)
84
Q

Features of types II and III cryoglobulinemia

A 
Mixed cryoglobulins (polyclonal IgG + mono- or polyclonal IgM)
Associated with HCV, connective disorders, lymphoproliferative disorders

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