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Malignant Heme Flashcards

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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
Mutations associated with ET
JAK2 (50%) Calreticulin MPL Gene
26
At what platelet count do you see hemorrhage with ET? And why?
>1.5 million (or with high dose ASA) Via acquired Von Willebrand Disease
27
When do you treat ET and with what?
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
28
Which MPN is LEAST likely to progress to myelofibrosis or AML?
ET
29
Which MPN has the worst prognosis?
Primary myelofibrosis
30
What is an odd way PMF can present?
GI bleed (complications of portal hypertension)
31
Treatment for PMF.
HLA Matched allogenic Hematopoietic Stem Cell Transplant If NOT a candidate, can use: Ruxolitinib (for splenomegy and hypercatabolic symptoms) Also consider hydroxyurea and splenectomy
32
Which cell type is increased in PMF that leads to marrow fibrosis?
Megakaryocytes (secrete excess fibroblast growth factor)
33
Causes of Hypereosinophilia
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
34
What eosinophil count is diagnostic of hypereosinophilic syndrome (HES)?
>1500/uL
35
Treatment for idiopathic HES
Glucocorticoids | Imatinib if associated platelet-derived growth factor mutation is present
36
Level of clonal abnormal cells present in bone marrow or blood to diagnose acute leukemia
20%
37
Most common acute leukemia in adults
AML
38
Smear findings in AML
Elevated leukocytes with low ANC, thrombocytopenia, anemia, myeloblasts (sometimes)
39
Cure rates for AML
35-40%- Adults younger than 60 | 5-15%- Adults >60
40
What are the unique features of APL?
Leukocytes with primary granules (mad coagulopathy) | Chromosomal translocation t(15;17)
41
How to you treat/cure APL?
ATRA
42
Name one good and one bad molecular marker for AML
Good: NPM1 (50%) Bad: FLT3-ITD (33%)- consider transplant in first remission
43
AML induction therapy
Anthracycline and cytarabine
44
AML response rate to induction
Complete response in 60-85% younger than 60
45
AML Consolidation considerations
Low risk: conventional chemo | High risk: allogenic Hematopoietic Stem Cell Transplant
46
Geriatric AML treatment
``` Supportive care/hospice Single agent chemo (oral hydroxyurea, low dose cytarabine) OR Hypomethalating Agents (decitabine, azacitidine) ```
47
What types of WBCs are involved in ALL in adults? How do these two types present?
B-Cell (75%)- extramedullary Disease (GI or testicular involvement) T-Cell - mediastinal mass with wheeze or skin involvement
48
What mutation can be seen in ALL?
Philadelphia chromosome (20-30%)
49
How does treatment change in ALL if the Philadelphia chromosome is present?
Need additional intensive consolidation (autologous or allogenic hematopoietic stem cell transplant) after treatment with TKI (ex imatinib)
50
Treatment for ALL
Induction: Vincristine, Anthracycline, Steroids, L-asparaginase *also need CNS prophylaxis Maintenance: oral mercaptopurine and methotrexate (for up to 2 years)
51
Side effects of L-asparaginase
Hypofibrinogenemia | Hypertriglyceridemia
52
Incidence of secondary cancer after childhood ALL
11% at 30 years (after radiation therapy)
53
EPO hemoglobin target ESRD
12g/dL
54
Indications for G-CSF
-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
Indication for autologous stem cell transplant
MM | Relapsed Aggressive Lymphoma
56
Who is the typical donor for Allogenic hematopoietic stem cell transplant?
HLA matched sibling
57
What percent of adults over 50 have MGUS?
3%
58
Initial workup for plasma cell dyscrasia
SPEP Serum immunofixation Serum free light chains
59
What should be used to look for lyric lesions in MM?
Skeletal survey (NOT bone scan)
60
Features of MM
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
Which features must you have to not biopsy a patient who has a monoclonal gammopathy?
IgG <1.5 g/dL Normal serum free light chains No end organ damage
62
Features of MGUS
M Protein <3 g/dL or Urinary M Protein (<500/24h) *if >1.5 high risk With <10% bone marrow clonal plasma cells
63
Difference between smoldering MM and smoldering WM?
Both with bone marrow clonal plasma cells (>10%) but Myeloma is IgA or IgG >\= 3 g/dL WM is IgM >\= 3 g/dL
64
When do you treat MM or WM?
M protein present with >10% bone marrow clonal plasma cells and disease specific symptoms
65
When do you say someone has multiple myeloma?
>\= 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
Signs and symptoms of WM.
Systemic: fatigue, b-symptoms, neuropathy, hyperviscosity, mucosal bleeding Physical exam: adenopathy, HSM Labs: anemia, thrombocytopenia
67
Lost the three types of MM
Non-IgM (1% progression to MM per year) IgM (1.5% progression to WM) Light chain (0.3% progression to PCD)
68
What is an abnormal free light chain ratio that increases risk of progression of smoldering myeloma?
<0.125 or >8
69
What is the percent conversion of smoldering myeloma to multiple myeloma?
10% per year for the first 5 years
70
MM Treatment
``` Induction Chemo (melphalan) Autologous Hematopoietic Stem Cell Transplant Observation/Maintenance ``` *all patients need bisphosphonates and pneumonia/flu shots!
71
Lenalidomide and Pomalidide side effect
VTE (need thromboprophylaxis)
72
Bortezomib side effect
``` Peripheral Neuropathy Herpes reactivation (need acyclovir) ```
73
Most common type of amyloidosis
AL Amyloid
74
Diseases associated with AL Amyloid
PCDs | Waldenström macroglobulinemia (rare)
75
Major organ system impacts of AL amyloid
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
Treatment of AL Amyloid
Autologous Stem Cell Transplant | Prognosis is dependent on cardiac involvement
77
What makes you ineligible for Autologous Stem Cell Transplant in AL amyloid? What are you treated with instead?
Age >70 Advanced cardiac disease Stage IV CKD Large effusions Get treated with Melphalan or Bortezomib based chemo
78
Diseases associated with AA amyloid
RA IND Familial Mediterranean Fever Chronic disease
79
What amyloid protein is deposited in Hereditary Amyloidosis
Mutated TTR or fibrinogen alpha chain
80
What type of amyloid protein is deposited in dialysis related amyloidosis?
Beta-2 microglobulin
81
Major complication of WM and how do you treat?
Hyperviscosity Syndrome Treat with plasmapheresis then targeted therapy with rituximab (+\- chemo) Steroids, *new TKI approved= ibrutinib
82
What is cryoglobulinemia?
Clonal or polyclonal Ig’s that precipitate at temp <37C and dissolve when rewarmed
83
Features of Type I Cryoglobulinemia
``` Monoclonal Immunoglobulin (IgM) Associated with Plasma cell dyscrasias Usually asymptomatic (May have ulcers, Raynaud phenomenon, digital ischemia) ```
84
Features of types II and III cryoglobulinemia
``` Mixed cryoglobulins (polyclonal IgG + mono- or polyclonal IgM) Associated with HCV, connective disorders, lymphoproliferative disorders ```

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