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Hematological Disease > Hematological Disease > Flashcards

Hematological Disease Flashcards

1
Q

What is AML with Normal Cytogenetics

A

Any non-lymphatic cell lineage can be affected. DNA sequencing required to determine if patients will respond to treatment.

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2
Q

AML Normal Cyto (Pharmacology)

A

Pharmacology:

  1. Treat if less than 60 and no comorbidity.
  2. Low Risk: Daunorubucin, cytarabine (maybe thioguanine)
  3. Gemtuzumab
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3
Q

AML Normal Cyto (Presentation)

A

Any age, pediatrics AML is uncommon, but happens Gingival Hypertrophy

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4
Q

AML Normal Cyto (Presentation for all AML)

A

Bruising, fatigue, bleeding, infection, bone pain

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5
Q

AML Normal Cyto (Immunophenotype)

A
  1. CD34+ + Lineage specific markers.
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6
Q

AML with Normal Cyto (Morphology)

A

Morphology: Undifferentiated

  1. Monocystic/monoblastic
  2. Vacuoles
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7
Q

AML t(8;21) (Translocation forms)

A

ETO-AML1 [RUNX1-RUNX1t1]

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8
Q

AML t(8;21) (Common ways translocation causes malignancy)

A
  1. Bind to DNA as co-repressor; inhibit transcription needed for differentiation
  2. Form constitutively active proproliferation system.
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9
Q

AML t(8;21) (Fulfills Gililand Hypothesis?)

A
  1. Unregulated proliferation (Class I)

2. Differentiation Inhibited (ClassII)

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10
Q

AML t(8;21) (Inhibits)

A

Inhibits Myeloid Differentiation

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11
Q

AML t(8;21) (Presentation)

A

Younger adults (age associated) and kids; pancytopenia symptoms (fatigue, infection, and bleeding).

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12
Q

AML t(8;21) (Immunophenotype)

A
  1. CD13+, CD33+ (Maturing myeloid)

2. CD34+ (Blasts)

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13
Q

AML t(8;21) (Morphology)

A

Morphology: Auer Rods

  1. Crystalized azurophilic granules (MPO); thus only in AML
  2. Large blasts with smooth/smudgy chromatin.
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14
Q

Acute Premyelocitic Leukemia (APL)

Translocation

A

t(15;17) causes fusion protein of transcription factor and retinoic acid receptor: PML-RARA

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15
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Gililand Hypothesis)

A

Fulfills Gililand Hypothesis: Inhibits granulocyte differentiation

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16
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Pharmacology)

A
  1. Induction: ATRA + AML Approved
  2. Consolidation: ATRA + Anthracycline
  3. Maintaince: ATRA + 6MP + Methotrexate
  4. Aresenic: BBW: Cardiovascular
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17
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Presentation)

A

Presentation: Thrombocytopenia, DIC, and leukocytosis.

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18
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Immunophenotype)

A

Immunophenotype:

  1. CD34-, HLA-DR- (Low Blasts, normal for APL
  2. CD13+, CD33+ (Immature myeloid markers)
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19
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Morphology)

A

Morphology: Big blasts

  1. Auer Rod Stacks.
  2. Heart-shaped/bat-wing nuclei + Butterfly Nuclei
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20
Q

Acute Premyelocitic Leukemia (APL) (t15;17) (Pharmacology)

A

Pharmacology:

  1. Induction: ATRA + Anthracycline
  2. Consolidation: Methotrexate + Mercaptopurine + ATRA
  3. Maintaince: Same as Consolidation
  4. Aresenic: BBW: Cardiovascular
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21
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Translocation forms)

A

Translocation forms: Fusion protein of transcription factor CBFB-MYH11

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22
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Fullfills Gililand Hypothesis?)

A

Fulfills Gililand Hypothesis

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23
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Inhibits)

A

Inhibits myeloid maturation. AKA” Core binding factor” Leukemia

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24
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Diagnostic Tools)

A

FISH diagnosis for inversion is backwards. Normally, fusion signals are bad, but for inversions they are normal.

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25
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Pharmacology)

A

Pharmacology:

  1. Treat if less than 60 and no comorbidity.
  2. Low Risk: Daunorubucin, cytarabine (maybe thioguanine)
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26
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Presentation)

A

Presentation: Kids, adults

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27
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Immunophenotype)

A

Immunophenotype:

  1. CD34+, CD117+ = blasts
  2. CD13+, CD33+ = maturing/granulocyte
  3. CD14+, CD11b+ = monocyte
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28
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Morphology)

A

Morphology:

  1. Mixed granulocyte-monocyte features(myelomonocytic)
  2. Eosinophilia
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29
Q

Acute Myelomoncytic Leukemia (AML) inv(16;16) t(16;16) (Prognosis)

A

Prognosis: poor, chemo improves.

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30
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Translocation forms)

A

Translocation forms p210 BCR-ABL1 [Philidelphia Chromosome] increased tyrosine kinase activity.

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31
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Stem cell affected)

A

Pluripotent stem cell affected (myeloid AND lymphoid)

— If granulocyte/monocyte –> Chronic mylelomonocytic Leukemia (CMML)

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32
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Blast Criss)

A 
Blast Criss (4 - 5 years):
--- Chronic phase --> accelerated phase --> Blast phase --> Leukemia (but blasts have no Auer rods)
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33
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Pharmacology)

A

Pharmacology:

  1. Acute phase - classical chemo
  2. Chronic phase - imatinib (2ndary: dadatininb, nilotinib)
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34
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Presentation)

A 
Presentation: 
-- 30 - 60 years (age related)
-- asymptomatic
PE: 
-- Hepatosplenomegaly (chronic disease)
Exposure:
-- Ionizing radiation + Benzene
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35
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (Morphology)

A

Morphology:

  1. BM Hypercellular
  2. BM No blasts (pre blast crisis)
  3. Unexplained basophilia (basophils) in PS
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36
Q

Chronic Myelogenous Leukemia (CML) t(9;22) (DDx)

A

DDx:

  1. RT-PCR of BCR-ABL1
  2. Very low LAP b/c non-functional leukocytes (vs. Leukomoid Rxn)
    * Leukomoid reaction: normal leukocytosis –> left shift; has high LAP
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37
Q

Mastocytosis (Genetics)

A

Mastocytosis Genetics:

  1. C-KIT mutation
  2. PDGF-RA via F1P1 translocation
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38
Q

Mastocytosis: Increased ______ _______ activity in mast cells (pathogenesis)

A

Mastocytosis: increased tyrosine kinase activity in mast cells

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39
Q

Mastocytosis (Where in the body does it present?)

A

Usually presents outside the bone marrow/lymph nodes

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40
Q

Mastocytosis (similar to _______ )

A

Mastocytosis is similar to Chronic Eosinophilic Leukemia (CEL)

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41
Q

Mastocytosis (presentation)

A

variable, confusing

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42
Q

Mastocytosis (Immunophenotype)

A
  1. Tryptase + (mast cell granule)

2. CD117+ (Blast CKIT, SCF-receptor), CD25+

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43
Q

Mastocytosis (Treatment)

A

Imatinib

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44
Q

Primary Mylelofibrosis (Etiology)

A

Jak2 Mutation on Ch.9 short arm - increased TK signaling via J/K pathway

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45
Q

Primary Mylelofibrosis (Pathogenesis)

A

Abnormal megs secrete cytokines –> deposition of Type 3 collagen –> marrow fibrosis + extra-medullary hematopoiesis (EMH) –> Fibrohematopoietic extra-med. tumors

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46
Q

Primary Mylelofibrosis (Pharmacology)

A
  1. Poor Rx treatment; splenectomy dangerous

2. HSCT is curative, but patients are too old

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47
Q

Primary Mylelofibrosis (Presentation)

A 
> 50 years old w/ splenomegaly
portal hypertension
osteosclerosis/bone pain
cytokine-like symptoms (muscle wasting)
Thrombosis
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48
Q

Primary Mylelofibrosis (Morphology)

A
  1. BM - full of type 3 collagen

2. Peripheral blood - bizarre megs, abnormal platelets, tear drop RBC, nucleated RBC

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49
Q

Primary Mylelofibrosis (DDx)

A

Reticulin Stains for Fibers

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50
Q

Primary Mylelofibrosis (Treatment)

A

Supportive care (blood transfusion)

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51
Q

Polycythemia Vera (Etiology)

A

Jak2 Mutation on Ch. 9 short arm - increased effect of growth factors (95%)

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52
Q

Polycythemia Vera (Pathogenesis)

A

Erythroid lineage is primarily affected > megs > others

Increased RBC/HCT –> sludgy RCB movement –> bad

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53
Q

Polycythemia vera vs. secondary polycythemia

A

PV is NOT secondary polycythemia (hypoxia/anemia induced EPO-related RBC production). That’s altitude sickness, atrial-septal defect, smoking, COPD, and Pickwickian syndrome.

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54
Q

Polycythemia Vera (Presentation)

A 
Thrombosis
CNS symptoms
Itchiness after hot bath
facies
blurred vision (retinal distention)
splenomegaly
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55
Q

Polycythemia Vera (Labs)

A

Decreased EPO (best to rule out reactive polycythemia)

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56
Q

Polycythemia Vera (Morphology)

A 
Erythroid hyperplasia (BM)
Increased RBC (PS)
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57
Q

Polycythemia Vera (Treatment)

A

Phlebotomy, deplete iron, aspirin, myelosuppressive

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58
Q

Polycythemia Vera (Prognosis)

A

Progress to MDS, AML, Myelofirbrosis

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59
Q

Essential Thrombocythemia (Genetics)

A

Jak2 Muations (50% of cases)

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60
Q

Essential Thrombocythemia (Pathogenesis)

A

Excess of dysplastic/abnormally functioning platelets from Megs

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61
Q

Essential Thrombocythemia vs. Secondary Thrombocythemia

A

Have to differentiate ET from Secondary Thrombocythemia:
– Bleeding, inflammation, iron deficiency, or asplenia will cause secondary thrombocytemia. Platelet count will not extremely high. TPO will be high.

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62
Q

Essential Thrombocythemia (Treatment/pharm)

A

Hydroxyurea

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63
Q

Essential Thrombocythemia (Presentation)

A

Thrombosis OR bleeding (GI), splenomegaly

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64
Q

Essential Thrombocythemia (Morphology)

A
  1. Increased megs, large/abnormal megs, clustering megs

2. Bizarre platelets = Meg Fragment

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65
Q

Essential Thrombocythemia (DDx)

A
  1. Extremely elevated platelets

2. Normal TPO

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66
Q

Essential Thrombocythemia (Prognosis)

A

10 yr survival, but can progress to myelofibrosis, MDS, acute leukemia

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67
Q

Myeloid Lineage Diseases - Name 4 classifications of Myelodysplastic Syndromes

A
  1. Refractory Cytopenia with Unilineage Dysplasia
  2. Refractory Anemia w/ ring sideroblasts
  3. MDS with Isolated del(5q)
  4. Refractory Cytopenia with Multilinage Dysplasia
  5. Refractory anemia with Excess Blast
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68
Q

Refractory Cytopenia with Unilineage Dysplasia (Etiology/Mechanism)

A
    • Clonally expanded acquired mutation of stem cell for one lineage
    • Monosomies/trisomies may be present
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69
Q

Refractory Cytopenia with Unilineage Dysplasia (Diagnosis)

A

Diagnosis is made on morphological findings: Megaloblastoid features; binucleated RBC w/ irregular nuclei; hypercellular marrow

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70
Q

Refractory Cytopenia with Unilineage Dysplasia (Presentation)

A

Unexplained cytopenia in elderly population (>65 yo)

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71
Q

Refractory Cytopenia with Unilineage Dysplasia (Immunophenotype)

A

Abnormal markers

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72
Q

Refractory Cytopenia with Unilineage Dysplasia (Morphology)

A

Megaloblastoid features; binucleated RBC w/irregular nuclei; hypercellular marrow

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73
Q

Refractory Cytopenia with Unilineage Dysplasia (Prognosis)

A

Survival normal for age

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74
Q

Refractory Anemia w/ ring sideroblasts

Etiology/Mechanism

A

Clonally expanded acquired mutation in erythroid cell line

Monosomies/trisomies may be present

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75
Q

Refractory Anemia w/ ring sideroblasts(Diagnosis)

A

Morphological findings + Iron studies

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76
Q

Refractory Anemia w/ ring sideroblasts(Morphology)

A

Ring sideroblasts with dyspoietic features in red cells only

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77
Q

Refractory Anemia w/ ring sideroblasts(Presentation)

A

Unexplained cytopenia iin elderly (>65yo)

Anemia + associated symptoms

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78
Q

Refractory Anemia w/ ring sideroblasts(Immunophenotype)

A

Abnormal markers

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79
Q

Refractory Anemia w/ ring sideroblasts(Prognosis)

A

Survival normal for age

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80
Q

MDS with Isolated del(5q)

Etiology/Mechanism

A

Loss of large arm of chromosome 5

ALL megakaryocytes fail to divide nuclei –> mononuclear megs

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81
Q

MDS with Isolated del(5q)

Presentation

A

Anemia (severe), elderly (>65), women

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82
Q

MDS with Isolated del(5q)

Morphology

A

All megs are mononucleated

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83
Q

MDS with Isolated del(5q)

Prognosis

A

Good survival
Treat with lenalidomide
10% progress to AML

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84
Q

Refractory Cytopenia with Multilinage Dysplasia (Etiology/Mechanism)

A

Clonally expanded acquired mutation in MULTIPLE cell lineages
50% show non-specific cytogenic abnormalities

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85
Q

Refractory Cytopenia with Multilinage Dysplasia (Morphology)

A

Granulocytes lacking normal granules; abnormal lobulation
– Morphology based upon lineages affected
If granulocytic expect abnormal granules
If erythroid, expect nRBC, etc.

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86
Q

Refractory Cytopenia with Multilinage Dysplasia (Presentation)

A

Anemia (severe), elderly (>65), women

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87
Q

Refractory Cytopenia with Multilinage Dysplasia (Immunophenotype)

A

Abnormal markers

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88
Q

Refractory Cytopenia with Multilinage Dysplasia (Prognosis)

A

Median survival 30 months

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89
Q

Refractory Anemia with Excess Blast (Genetics)

A

RAEB-1: 5 - 9% morphological blasts
RAEB-2: 10 - 19% morphological blast
50% show nonspecific abnormal cytogenics

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90
Q

Refractory Anemia with Excess Blast (Pathogenesis)

A

> 20% –Acute leukemia

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91
Q

Refractory Anemia with Excess Blast vs. Acute Leukemia

A

AMLs develop basts from translocated mutation that halts differentiation. Here, a mutation occurs that is clonally expanded and takes over as a cell line. This is more like a TRUE malignancy.

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92
Q

Refractory Anemia with Excess Blast (Presentation)

A

Cytopenia in elderly patient (65+yo)

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93
Q

Refractory Anemia with Excess Blast (Immunophenotype)

A
  1. CD34+

2. CD117+

94
Q

Refractory Anemia with Excess Blast (Morphology)

A

Blasts and dyspoeitic maturation

95
Q

Refractory Anemia with Excess Blast (Prognosis)

A

RAEB-1 – 25% progression to AML

RAEB-2 –33% progression to AML

96
Q

Name the 5 Acute Lymphoid Leukemias (ALL)

A
  1. B-Cell ALL t(9;22)
  2. B-Cell ALL t(11;19)
  3. B-Cell ALL t(12;21)
  4. Hairy Cell Leukemia
  5. T-Cell ALL
97
Q

B-Cell ALL t(9;22) – Etiology/Mechanism

A

Fusion protein of BCR-ABL 1

  1. Fusion protein of serine-threonine kinase (BCR) to tyrosine kinase (ABL1) — equivalent to Class I mutation
  2. IKZF1 transcription factor inhibited (Differentiation inhibited) — equivalent to Class II mutation
98
Q

B-Cell ALL t(9;22) vs. CML t(9;22)

A

Different than CML t(9;22);

  1. Different break in oncogene
  2. Different fusion protein
99
Q

B-Cell ALL t(9;22) – (Pharmacology)

A
  1. Induction: Anthracycline, vincristine, prednisone
  2. Maintenance: methotrexate + 6MP
  3. CNS (intrathecal) prophylaxis: methotrexate
100
Q

B-Cell ALL t(9;22) – (Presentation)

A

Kids and older adults

101
Q

B-Cell ALL t(9;22) – (Immunophenotype)

A

Immunophenotype: (early B cell)

  1. CD10+
  2. CD19+
  3. TdT+
102
Q

B-Cell ALL t(9;22) – (Morphology)

A

Big agranular blasts

103
Q

B-Cell ALL t(9;22) – (Prognosis)

A

poor prognosis

104
Q

B-Cell ALL t(9;22) – (Treatment)

A
  1. Induction: Anthracycline, vincristine, prednisone
  2. Maintenance: methotrexate + 6MP
  3. CNS (intrathecal) prophylaxis: methotrexate
    Note: This is tyrosine so imatinib is also and option
105
Q

B-Cell ALL t(11;19) – (Etiology)

A

Rearranged MLL

  1. Fusion of transcription regulator (histone methyl transferase) to many chromosomes (Class II)
  2. FLT3 mutations (Class II)
106
Q

B-Cell ALL t(11;19) – (Mechanism)

A

MLL becomes transcriptional repressor –> decrease differentiation

107
Q

B-Cell ALL t(11;19) – (Prognosis)

A

Poor prognosis

108
Q

B-Cell ALL t(11;19) – (Presentation)

A

Kids <1yo, most common form in very young children

109
Q

B-Cell ALL t(11;19) – (Immunophenotype)

A

Early B cell

  1. CD10- (differentiate between t(9;22)ALL)
  2. CD19+
  3. TdT+
110
Q

B-Cell ALL t(11;19) – (Morphology)

A

Big agranular blasts

111
Q

How do you differentiate the immunophenotypes of B-Cell ALL t(9;22) and B-Cell ALL t(11;19)?

A

B-Cell ALL t(9;22) = CD10+

B-Cell ALL t(11;19) = CD10-

112
Q

B-Cell ALL t(12;21) – Etiology/Mechanism

A

Fusion protein of TEL-AM1 [ETV6-RUNX1]
1. Dominant negative transcription factor (Class II)
2. Pax5 Mutation inhibits differentiation (Class II)
Gilliand hypothesis not supported

113
Q

B-Cell ALL t(12;21) – Prognosis

A

Good - 90% cure

114
Q

B-Cell ALL t(12;21) – Presentation

A

Kids - 25% of all prediatric B-ALL

115
Q

B-Cell ALL t(12;21) – Immunophenotype

A
  1. CD34+
  2. CD20-
  3. TdT+
116
Q

B-Cell ALL t(12;21) – Morphology

A

Big agranular blasts

117
Q

Hairy Cell Leukemia (Etiology)

A

BRAF V600F mutation

118
Q

Hairy Cell Leukemia vs. all other types of leukemia

A

Only leukemia without lymphadenopathy

119
Q

Hairy Cell Leukemia (Key Diagnosics)

A

Resistant to Tartrate-resistant acid phosphatase (TRAP)

120
Q

Hairy Cell Leukemia (Presentation)

A

Thrombocytopenia
Splenomegaly
Dry bone marrow
*Common in middle aged men

121
Q

Hairy Cell Leukemia (Immunophenotype)

A

CD19
CD20
B cell marker

122
Q

Hairy Cell Leukemia (Treatment)

A

Rituximab
Interferon alpha 2-a, b
Cladribine
Pentostatin

123
Q

T-Cell ALL (Etiology)

A

Oncogenic translocation to Ch.14

124
Q

T-Cell ALL (Mechanism)

A

Oncogene is translocated to Ig or TCR promoter on Ch 14

125
Q

T-Cell ALL (Prognosis)

A

High risk

126
Q

T-Cell ALL (Presentation)

A

Kids - 25% of all pediatric
Thymic, lymph mass
Splenomegally (where T cells go)

127
Q

T-Cell ALL (Immunophenotype)

A
  1. CD3+ CD5+ (T cell markers)
  2. TdT+ (marker f T/B cell precursors)
  3. B-cell/myeloid CDs/ antigens
128
Q

T-Cell ALL (Morphology)

A

Big agranular blasts

129
Q

Classical Hodgkin’s Lymphoma (Etiology)

A

Three fold etiology:

  1. No Ig expressison
  2. Anti-apoptosis (via NFkB or EBV)
  3. Genetically unstable, more mutations
130
Q

Classical Hodgkin’s Lymphoma (Affected Cell)

A

B-Lymphocye –> R-S Cell

131
Q

Classical Hodgkin’s Lymphoma (4 Types)

A
  1. Nodular lymphocyte predominant (best prognosis)
  2. Nodular sclerosing (women>men)
  3. Mixed cellularity (most ass. w/ EBV)
  4. Leukocyte depleted (worst prognosis)
132
Q

Classical Hodgkin’s Lymphoma (Presentation)

A 
Older men (except nodular sclerosing)
cervical adenopathy
B-like symptoms
rare extranodal involvement
EBV infection
133
Q

Classical Hodgkin’s Lymphoma (Immunophenotype)

A 
Lymphocyte dominant HL
1.  CD15.CD30 - (few RS cells)
2.  CD20+
3.  Pax5+
All other HL types
1.  CD15/30+ = RS Cells
2.  CD20+
3.  Pax 5+
134
Q

Classical Hodgkin’s Lymphoma (Morphology)

A

RS cells over diverse cellular backdrop

135
Q

Classical Hodgkin’s Lymphoma (Treatment)

A

ABVD

136
Q

Nodular Lymphocyte Dominant Hodgkin’s Lymphoma (Etiology)

A
  1. Ig is EXPRESSED
  2. Anti-apooptosis (NFkB, EBV)
  3. Genetically unstable, more mutations
137
Q

Nodular Lymphocyte Dominant Hodgkin’s Lymphoma (Affected cell)

A

B-cell –> lympho-histocytic (L/H variant)

138
Q

Nodular Lymphocyte Dominant Hodgkin’s Lymphoma (Prognosis)

A

Best prognosis of all HL types

139
Q

Nodular Lymphocyte Dominant Hodgkin’s Lymphoma (Immunophenotype)

A
  1. CD15.CD30 - (few RS cells)
  2. CD20+
  3. Pax5+
140
Q

Nodular Lymphocyte Dominant Hodgkin’s Lymphoma (Morphology)

A

RS cells with collar of T-Lymphocytes

Popcorn cell

141
Q

Name 8 classifications of non-Hodgkin’s Lymphoma

A
  1. Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma
  2. Mantle cell Lymphoma
  3. Follicular Lymphoma
  4. Diffuse Large B Cell Lymphoma
  5. Burkitt’s Lymphoma
  6. Angioimmunoblastic T-Cell Lymphoma
  7. Peripheral T-Cell Lymphoma not otherwise specified
  8. Mycosis Fungiocides and Sezary Syndrome
142
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

4 potential etiologies

A
  1. Deletion in chromosome 13 (good prognosis)
  2. Trisomy 12
  3. Deletion in Chromosome 11
  4. Deletion in Chromosome 17 (p53 - bad prognosis)
143
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Big complication

A

Hypogammaglobulinemia - look for recurrent infections

144
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Affected cell

A

Memory cell

145
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Presentation

A 
Older men w/ recurrent infections (hypogammaglobulinemia)
Warm helolytic anemia
Diagnostic features based on staging:
0 - lymphocytosis
1 - nodal involvement, both sides of diaphragm
2 - hepatosplenomegaly
3 - <10Hg (anemia)
4 - thrombocytopenia
146
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Immunophenotype

A

Light chain restricted (clonal)

  1. C 20+ (weak)
  2. CD 5+ (odd, but characteristic phenotype of CLL/SLL)
    * ZAP-70 expression is bad
    * CD38 expression is bad
147
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Lymph node biopsy

A

Effaced lymph node = Pseudofollicular

148
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Peripheral smear

A

Small lymphocytes, SMUDGE CELLS

149
Q

Chronic Lymphocytic Leukemia/Chronic Lymphocytic Lymphoma

Treatment

A

IV Ig; Combination (rituximab, fludrabine)

150
Q

Mantle Cell Lymphoma (Etiology)

A

t(11,14) - CyclinD1-IgH

151
Q

Mantle Cell Lymphoma (Mechanism)

A

Affected Cell: B cell in (pregerminal center) mantle zone or memory cell in periphery
Cyclin D1 expression pushes cells: G1 –> s

152
Q

Mantle Cell Lymphoma (Presentation)

A

Similar presentation to CLL; worst prognosis

153
Q

Mantle Cell Lymphoma (Immunophenotype)

A

Light chain restricted

  1. CD20+ (strong)
  2. CD5+
  3. K1g7+ = marker for fast growth
154
Q

Mantle Cell Lymphoma (Lymph node biopsy)

A

Effacement = Starry Sky

155
Q

Mantle Cell Lymphoma (Peripheral blood stain)

A

SMUDGE CELLS

156
Q

Mantle Cell Lymphoma (Treatment)

A

Combination (Fludrabine + Rituximab; CVP, CHOP, CHOP-R)

157
Q

Follicular Lymphoma (Etiology)

A

t(14;18) IgH-BCL-2

158
Q

Follicular Lymphoma (Grading)

A

Grade 1: shows lots of centrocytes > least aggressive
Grade 2: mixture of centrocytes/centroblasts
Grade 3: lots of centroblasts > aggressive

159
Q

Follicular Lymphoma (Presentation)

A

older, w/painless lymphadenopathy (if painful = reactive (flu))

160
Q

Follicular Lymphoma (Lymph node biopsy)

A

Follicular hyperplasia
Effacement of architecture
slow mitosis + few tingible body macrophages (indolent, slow growing)

161
Q

Follicular Lymphoma (Immunophenotype)

A
  1. CD19+, CD20+ (B cell)
  2. CD 10+
  3. BCL-2
162
Q

Follicular Lymphoma (DDx)

A

Immunohistochemistry + immunophenotyping (BCL-2 stain)

163
Q

Follicular Lymphoma (Prognosis)

A

See grade: progress to diffuse large B cell lymphoma

164
Q

Diffuse Large B Cell Lymphoma (Etiology)

A

T(V;3) - BCL-6

t(14;18) - BCL2-IgH

165
Q

Diffuse Large B Cell Lymphoma (Is it common?)

A

Most common NHL in Adults; poorly defined group

166
Q

Diffuse Large B Cell Lymphoma vs. Follicular Lymphoma

A
  1. Extranodal involvement

2. RAPID DIVISION

167
Q

Diffuse Large B Cell Lymphoma (Presentation)

A 
Older patients (few pediatric cases)
Most common NHL in Adults
168
Q

Diffuse Large B Cell Lymphoma (Immunophenotype)

A
  1. CD19+, CD20+
  2. CD10+
  3. BCL-6/BCL-2+
169
Q

Diffuse Large B Cell Lymphoma (Lymph node biopsy)

A

Immature (Large B) cells with high mitotic rate

170
Q

Diffuse Large B Cell Lymphoma (Treatment)

A

CHOP + R-CHOP

171
Q

Burkitt’s Lymphoma (Etiology)

A

t(8;14)
t(2;8)
t(8;22)
MYC gene + IgH, IgL, IgK

172
Q

Burkitt’s Lymphoma (African Burkitts vs. Sporadic case)

A

African Burkitt’s: Malaria belt, EBV

Sporadic case: Everywhere else, NOT EBV associated

173
Q

Burkitt’s Lymphoma (Presentation)

A

Endemic: Jaw Lesions
Sporadic: Abdomen or pelvic lesions

174
Q

Burkitt’s Lymphoma (Immunophenotype)

A
  1. CD19+, CD20+
  2. CED22+
  3. CD79+
  4. Ki-67+
175
Q

Burkitt’s Lymphoma (Morphology)

A

Starry Sky appearance w/ sheets of lymphocytes/blasts containing empty spaces of tingible body macrophages

176
Q

Burkitt’s Lymphoma (Treatment)

A

Usually curable in children; long term care not likely

177
Q

Angioimmunoblastic T-Cell Lymphoma (Etiology)

A

Clonal expansion of abnormal T-Helper cells in pericortical region of lymph node

178
Q

Angioimmunoblastic T-Cell Lymphoma (Key diagnostic feature)

A

CD10+ Immunophenotype

179
Q

Angioimmunoblastic T-Cell Lymphoma (CD10+ in germinal center. What is the cell type?)

A

B-cell

180
Q

Angioimmunoblastic T-Cell Lymphoma (CD10+ inbetween follicles. What is the cell type?)

A

T-cell

181
Q

Angioimmunoblastic T-Cell Lymphoma (Affected cell)

A

CD4 Follicular T-cell

182
Q

Angioimmunoblastic T-Cell Lymphoma (Presentation)

A

Rapid critical illness
Adenopathy
Hepatosplenomegaly
Compromised immunity

183
Q

Angioimmunoblastic T-Cell Lymphoma (Immunophenotype)

A

CD3+
CD4+
CD10+

184
Q

Angioimmunoblastic T-Cell Lymphoma (Morphology)

A

Expansion of pericortical area

  1. Prominent vessels - look for eosinophilia
  2. Effacement of overall architecture
  3. Immunoblasts
185
Q

Peripheral T-cell Lymhoma Not otherwise specified (Definition)

A

Poorly defined group of AGGRESSIVE T-cell Lymphoma

186
Q

Peripheral T-cell Lymhoma Not otherwise specified (Prognosis)

A

Aggressive; survival <5 years

187
Q

Peripheral T-cell Lymhoma Not otherwise specified (Presentation)

A 
Lymphadenopathy
Paraneoplastic syndromes (hemolytic anemia
B-type syndrome
188
Q

Peripheral T-cell Lymhoma Not otherwise specified (General Immunophenotype)

A

T-cell markers + abnormal markers (B-cell, random)

189
Q

Peripheral T-cell Lymhoma Not otherwise specified (Lymph node biopsy)

A

Cells too uniform for the lymph node

  1. Expanded paracortex
  2. Effacement of architecture
  3. Paracortical Epithelioid Histocytes
190
Q

Mycosis Funciosides and Sezary Syndrome (Affected cells)

A

Both involve Neoplastic CD4 T Cells

191
Q

Mycosis Funciosides and Sezary Syndrome (Definition)

A

Sezary syndrome is mycosis fungiosies with leukemic phase - circulating cells are called Sezary cells

192
Q

Mycosis Funciosides and Sezary Syndrome (Presentation)

A

Skin rash
Pruritis
Psoriasis
Neoplastic cells in the epidermis are called Putrier’s microabcesses

193
Q

Name 4 other diseases of Bone Marrow

A
  1. Aplastic Anemia
  2. Multiple Myeloma
  3. Waldenstrom’s Hyogammaglobulinemia
  4. Amyloidosis
194
Q

Aplastic Anemia (Primary cause)

A

Of all the causes, autoimmune destruction of stem cells is the PRIMARY CAUSE OF APLASTIC ANEMIA

195
Q

Aplastic Anemia (Etiologies)

A

Reduction or deletion of hematopoietic stem cells because:

  1. Cells are damages
  2. Immunosuppression of hematopoiesis
  3. Bad BM environment
196
Q

Aplastic Anemia (Congenital vs. Acquired etiologies)

A

Congenital: Faconi’s + Familial Aplastic
Acquired: Benzene, ionizing radiation, infections, PNH

197
Q

Aplastic Anemia (Important point)

A

Aplastic Anemia is NOT a malignancy

It is anemia cause by BM failure

198
Q

Aplastic Anemia (Presentation)

A

Variable
Anemic (normocytic)
Bleeding
Infections

199
Q

Aplastic Anemia (Labs)

A

Pancytopenia
Maybe leukocytosis (relative)
Decreased reticulocyte count (BM failure)

200
Q

Aplastic Anemia (Morphology of BM)

A

Hypocellular

Yellow Marrow

201
Q

Aplastic Anemia (Treatment)

A

Remove causative agent
Supportive care
Immunosupressive
HSCT

202
Q

Multiple Myeloma (Definition)

A

Abnormal plasma cell clonal expansion leading to multiple lytic bone lesions

203
Q

Multiple Myeloma (Etiology/Pathogenesis)

A

Bone lesions caused by myeloma cell production of DKK1 –> IL6 –> RANK-L –> stimulates osteoclasts, inhibits osteoblasts

204
Q

Multiple Myeloma (Stages)

A

Stage 1 - Normal Bence Jones; Hg, few lesions
Stage 2 - not 1 or 3
Stage 3 - Lots of Bence Jones, multiple lesions, anemic

205
Q

Multiple Myeloma (Presentation)

A 
Old
African American
Arthritis
Pain
CRAB  
Cytopenia (plasma cell infiltration)
Low Ig levels (other than IgG)
206
Q

Multiple Myeloma (DDx)

A

Serum plasma E-Phoresis –> Immunofixation

|&raquo_space; M-Spike (monoclonal protein)

207
Q

Multiple Myeloma (Immunohenotype)

A

CD38
CD138 (Plasma Cell)
CD19
CD20

208
Q

Multiple Myeloma (Peripheral smear)

A

Rouleax formation in blood

209
Q

Multiple Myeloma (Bone marrow)

A

Holes in bones

Packed w/ irregular plasma cells

210
Q

Multiple Myeloma (Prognosis)

A

Deletion of Ch.17 is key genetic (negative) predictor

211
Q

Multiple Myeloma (Treatment)

A

Bortezumib (proteosomes), lanilomide (immunomodulator), HSCT

212
Q

Waldenstrom’s Hyogammaglobulinemia (Etiology/Mechanism)

A

Lymphoma producing excess IgM paraprotein caused by maturation arrest etween mature B-cell and plasma cell

213
Q

Waldenstrom’s Hyogammaglobulinemia (Is it myeloma or lymphoma?)

A

It acts like myeloma, but as a mass they are lymphoma

214
Q

Waldenstrom’s Hyogammaglobulinemia (Presentation)

A

Hyperviscosity syndrome becaue of pentameric IgM

NO BONY DESTRUCTION

215
Q

Waldenstrom’s Hyogammaglobulinemia (DDx)

A

SPEP, Immunofixation

216
Q

Waldenstrom’s Hyogammaglobulinemia (Treatment)

A

Rituximab
Borezamib
Lanalidomide

217
Q

Amyloidosis (Definition)

A

Abnormal protein caused by light chain build up

218
Q

Amyloidosis (Etiology/Associated diseases)

A

Chronic inflammation
Familial (Transtyretin)
Secondary to myeloma

219
Q

Amyloidosis (Presentation)

A 
Any organ affected by amyloid
- Heart
- Liver
- Kidney
Macroglossitis - swollen tongue
Neuropathies
220
Q

Amyloidosis (DDx)

A

CONGO RED STAIN –> APPLE RED BIREFRINGENCE

221
Q

Amyloidosis (Treatment)

A

Borizamib
Lanilomide
*If familial (transthyretin) –> bone marrow transplant

222
Q

Name 5 General Characteristics of T Cell Lymphomas

A
  1. Not usually caused by translocation (except ALK + Anaplastic Large Cell Lymphoma - t(2;5))
  2. Early odd number CDs
  3. Thymus: TdT+, cytoplasmic CD3, CD4 and CD8
  4. Peripheral: CD4 or CD8, surface
    CD3
  5. Determine clonality
223
Q

Name 4 General Characteristics of Lymph Node Malignancies

A
  1. These are all centroblasts or centrocytes (centroblasts are worse b/c less differentiated)
  2. Clonal expansion of centroblasts or centrocytes that have evaded apoptosis in the lymph node by upregulating BCL-2/BCL-6
  3. Fewer tingible body macrophages
224
Q

Name the 3 Large categories of Hematological Diseases

A
  1. Myeloid Lineage (Myeloid, Erythroid, Megs)
  2. Lymphoid Lineage (B, T and NK cells)
  3. Other Bone Diseases
225
Q

Hematological Diseases > Name the 3 categories of Myeloid Lineage Diseases

A
  1. Acute Myeloblastic Leukemia
  2. Chronic Myeloproliferative Diseases
  3. Myelodysplastic Syndrome
226
Q

Hematological Diseases > Outline general mechanisms for each of the 3 categories of Myeloid Lineage Diseases

A
  1. Acute Myeloblastic Leukemia
    - – Clonal expansion of abnormal immature cells = BLASTS
  2. Chronic Myeloproliferative Diseases
    - – Clonal expansion of differentiated cell types
  3. Myelodysplastic Syndrome
    - – Either too few or expansion of ineffective cell types
227
Q

Hematological Diseases > Name the cell types associated with Lymphoid Lineage

A

B, T and NK cells

228
Q

Hematological Diseases > Lymphoid Lineage > Name the 2 categories of disease

A

Leukemia and Lymphoma

229
Q

Hematological Diseases > Outline general mechanisms for the 2 categories of Lymphoid Lineage Diseases

A

Leukemia
— Clonal expansion of abnormal immature cells = BLASTS
Lymphoma
— Clonal expansion of abnormal cell type within lymph tissue, mostly node

230
Q

Hematological disease > name the 4 types that do not fall into Myeloid or Lymphoid lineages

A

Aplastic Anemia
Multiple myeloma
Waldenstrom’s Macroglobulinemia
Amyloidosis

Hematological Disease (3 decks)

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