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SBL Final > Clinical > Flashcards

Clinical Flashcards

1
Q

Benzene is assoc with:

A

AML

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2
Q

TQ

Radiation may cause all leukemias except:

A

CLL

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3
Q

TQ
Describe the chromosomal damage seen in CML.
What is the translocation?
What is the result?

A

CML:

  • Philadelphia chromosome
  • t (9 : 22)
  • bcr joins abl&raquo_space; new “fusion gene with tyrosine kinase activity
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4
Q

t (15 : 17) is assoc with:

A

Acute Promyelocytic Leukemia (APL)

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5
Q

In the acute leukemias (especially ALL) patients often complain of:

A

Bone pain

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6
Q

Which leukemia, acute or chronic, is the dx often suspected on the basis of a CBC done as part of a routine workup for another problem?

A

Chronic leukemias.

CBC catches it even though the pt is feeling great.
Most pts with chronic leukemias are asymptomatic at time of dx.

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7
Q

What 3 diagnostic symptoms are you most likely to see in AML?

A
  • Renal failure
  • Hyperuricemia
  • Azotemia
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8
Q

2 chemicals labeled as causative factors of AML:

A

Benzene

Toluene

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9
Q

Which 2 subtypes of AML are more likely to have CNS involvement?

A

M4 and M5

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10
Q

Which 2 subtypes of AML are more likely to involve the skin (Sweet’s syndrome)?

A

M4 and M5

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11
Q

TQ

Gingival hypertrophy from infiltration by myeloblasts occurs in:

A

M4 and M5

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12
Q

What is the MC cytogenetic translocation seen in M2?

A

t (8 ; 21)

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13
Q

t (15 : 17) is pathognomonic for what AML subtype?

A

M3

acute promyelocytic leukemia; APL

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14
Q

TQ

  • MC malignancy in childhood
  • Whites, males
  • Assoc with HTLV-1 ***
A

Acute lymphocytic leukemia (ALL)

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15
Q

TQ

T-cell leukemias have a higher incidence of mass in what specific location?

A

Mediastinal masses (thorax)

Rule of T’s:

  • T-cell leukemias
  • Located on Thorax (mediastinum)
  • HTLV-1
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16
Q

Leukostasis occurs more frequently in:

A

AML

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17
Q

Lymph node and splenic enlargement are more common in:

A

ALL

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18
Q

How does age factor into prognosis of ALL?

A

Young age assoc with better prognosis (e.g., children 2 to 10 years of age do best)

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19
Q

TQ

In the treatment of AML, what is given in the induction phase?

A

3 + 7 (Anthracycline (Donarubicin or Inarubicin) and Ara-C)

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20
Q

TQ

In the treatment of AML, what is given in the consolidation phase?

A

High dose Ara-C (HDAC)

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21
Q

TQ

In the treatment of AML, what is given in the maintenance phase?

A

ATRA

-for APL (M3) ONLY!

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22
Q

TQ

In the treatment of ALL, what is given in the induction phase?

A

Vincristine/Prednisone + others

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23
Q

TQ

In the treatment of ALL, what is given in the consolidation phase?

A

Multiple agents + CNS prophylaxis (MTX or Ara-C)

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24
Q

TQ

In the treatment of ALL, what is given in the maintenance phase?

A

6-MP and MTX

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25
Q

TQ

  • An acquired clonal disorder involving the hematopoietic stem cell characterized by a prominent expansion of the granulocyte compartment
  • Cause: increases with radiation ***
  • MC in 5th and 6th decade, although childhood cases have been reported
A

Chronic myelocytic leukemia (CML)

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26
Q

TQ

The chronic phase of CML is characterized by:

A

Leukocytosis and indolent disease course

  • Chronic phase is 5-7 years
  • Most cells in blood are mature granulocytes
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27
Q

TQ

The accelerated phase of CML is characterized by:

A
  • Increased leukocytosis
  • Immature granulocytes appear in peripheral blood
  • Platelet and RBC counts begin to drop
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28
Q

TQ
The blast phase of CML is morphologically similar to AML, except that it is:
What are 2 prominent features of CML?
10% of pts who develop blast crisis will also have what?

A

Resistant to treatment

  • Anemia and thrombocytopenia prominent
  • 10% of pts who develop blast crisis will also have a lymphoid crisis (will also develop ALL due to it being a stem cell abnormality)
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29
Q

TQ

What is the MC cytogenetic translocation seen in CML?

A

Ph; t (9 ; 22) (q34 ; q11)

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30
Q

TQ

A decreased leukocyte alkaline phosphatase (LAP) score is diagnostic of:

A

CML

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31
Q

TQ

The initial treatment of choice of CML is:

A

TKIs

BCR/abl tyrosine kinase inhibitors

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32
Q 
TQ
What are the goals and benchmarks for treatment of CML with a TKI?
3-6 mo:
6 mo:
12 mo:
18 mo:
A

3-6 mo: complete hematologic response
6 mo: any cytogenetic response
12 mo: major cytogenetic response (50-75% reduction)
18 mo: complete cytogenetic response (cannot see anything resembling CML whatsoever)

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33
Q

TQ

  • MC chronic leukemia
  • Affects those ages 60+
  • Males more common
  • B-cell origin more common
A

Chronic lymphocytic leukemia (CLL)

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34
Q

What are a few associated abnormalities with CLL?

Think anemia-related…

A
  • 10-20% of pts have Coombs positive hemolytic anemia
  • Abs usually warm-reacting IgG
  • 95% of cases have immunoglobulin coating the surface of CLL cells (usually IgM if on CLL cells)
  • Hypogammaglobulinemia (due to intrinsic defect in B lymphocyte)
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35
Q

TQ
CLL can develop into what other neoplasm?
What syndrome is this called?

A

CLL&raquo_space; Large cell lymphoma

Richter’s syndrome

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36
Q

TQ

“Smudge cells” are seen in the peripheral smear of:

A

CLL

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37
Q

Dx:
What is the link between B cell and T cell neoplasms in terms of surface antigens? (i.e., why can CLL turn into large cell lymphoma?)

A

CD5

B cell markers = CD19, CD20, CD23
-Co-expression of CD5 in CLL (aberrantly expressed)

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38
Q

TQ
Which translocation is assoc with a worse prognosis in CLL?
What is the most frequent abnormality encountered?

A

t (11 ; 14) – translocates a gene called bcl-1 next to a gene regulating immunoglobulin production, assoc with worse prognosis

Most frequent abnormality encountered in CLL = Trisomy 12

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39
Q

TQ

Describe the Rai staging system for CLL (stages 0–IV).

A

Stage 0: Lymphocytosis only (ALC > 10,000/mm3) – 150 mo survival
Stage I: + LAD – 101 mo survival
Stage II: + hepatosplenomegaly – 71 mo survival
Stage III: + anemia (Hb < 11 gm) – 22 mo survival
Stage IV: + thrombocytopenia (< 100,000) – 11 mo survival

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40
Q

Increased fungal sepsis is seen in which chemotherapeutic drug used for B-CLL?

A

Fludarabine

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41
Q

TQ

What virus reactivation should you be aware of with ANY anti-CD20 monoclonal Ab (ofatumumab, obinituzumab)?

A

Hepatitis B

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42
Q

Treatment is not needed for CLL pts in which Rai stages?

A

Tx not needed for stages 0, I or II

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43
Q

What is the 3-drug treatment regimen for younger pts with CLL?

A

Fludarabine / Cytoxan / Rituxan

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44
Q

What are the 2 treatment regimen options for older pts with CLL?

A

Chlorambucil +/– Prednisone
or
Bendamustine / Rituxan

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45
Q

For the severely ill pt with CLL, what is the treatment?

A

NO treatment.

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46
Q

What are the two major categories of HL?

A
  • Classical

- Nodular lymphocyte predominant HL

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47
Q 
TQ!
Which form of classical HL has the worst prognosis?
A. Lymphocyte predominant
B. Nodular Sclerosis
C. Mixed Cellularity
D. Lymphocyte depleted
A

D. Lymphocyte Depleted

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48
Q

What are the two patterns of spread for HL?

-Usually starts as focal microscopic disease in a peripheral lymph node (usually cervical)

A

-Contiguity model: local process and spreads to contiguous lymph nodes, hematogenous spread occurs late

-Susceptibility model: systemic disease from infection by an oncogenic virus


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49
Q

Pt with constitutional HL presents with…

  • Fatigue, weakness, anorexia, cachexia, pruritis
  • Lymph node pain after EtOH ingestion
  • Impaired immunity
  • B symptoms

TQ
What are B symptoms? Why are they impt?

A
  • Weight loss > 10% of body wt. in 6 months prior to dx
  • Drenching night sweats
  • Unexplained fever with temp. > 38oC

Associated with 20-30% reduction in survival

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50
Q

What kind of biopsy do we need in HL?

A

excisional NOT FNA of LN

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51
Q

How do we stage HL using the Ann Arbor Staging System? (Hint 4 stages)

A

I: Single LN
II. 2+ LN on same side of diaphragm
III. LN on both sides of diaphragm
IV: Many LN or BM involvement

All cases sub-classified into absence (A) or presence (B) of B symptoms

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52
Q

TQ

What tx do we use for pts with stage I or II HL?

A

Radiation therapy ! curative!

w/ chemo in 3-4 cycles for high risk or bulky dz

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53
Q

TQ

When do we use chemo in HL? What does it consist of?

A
  • Stage III or IV
  • ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine)

(w/ radiation for residual dz)

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54
Q

SE of chemo using ABVD for HL?

A
  • cardiotoxicity
  • pulmonary toxicity (bleomycin)
  • Neutropenia, thrombocytopenia
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55
Q

TQ

How do we treat large mediastinal masses in HL?

A

chemotherapy followed by radiation

cant do initial radiation because too large a field–>risky

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56
Q

TQ

How do we treat relapses in HL?

A
  • modality other than that used for primary tx

- BM transplant?

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57
Q

What are the three general categories of lymphoma based on untreated survival?

A
  • Indolent: survival measured in years
  • Aggressive: survival measured in months
  • Highly Aggressive: survival measured in weeks
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58
Q

TQ!
Pt on graft-rejection medications for transplant. Presents with acute altered mental status, seizures, confusion
MRI shows big lesion in brain

A

Primary CNS lymphoma as result of chronic immunosuppression

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59
Q

Higher incidence of lymphoma in what 3 pt groups?

A
  • AIDS
  • transplant recipients
  • genetic immunodeficiency patients (Wiskott-Aldrich syndrome, X linked agammaglobulinemia, etc.)
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60
Q

TQ

  • often asymptomatic, disease discovered incidentally on evaluation for something else
  • B symptoms may be present
  • Infrequently present with peripheral adenopathy, but may have massive mediastinal or retroperitoneal node involvement
A

Indolent lymphoma

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61
Q

TQ
-history of waxing and waning adenopathy for several weeks or months
-Constitutional symptoms (fatigue, weakness, weight loss) more often
Splenomegaly

A

Aggressive lymphoma

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62
Q

TQ

  • rapid development of adenopathy and symptoms
  • Frequently present with an isolated lesion that “came up overnight”
A

Highly aggressive lymphoma

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63
Q

TQ
Indolent lymphomas often present as which stage?
What about aggressive and highly aggressive?

A
  • Indolent lymphomas often present with advanced (Stage III or IV) disease
  • Aggressive and highly aggressive lymphomas frequently present with lower stage (Stage I or II) disease
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64
Q

TQ

What is richter’s syndrome?

A

large cell lymphoma arising from chronic lymphocytic leukemia (CLL)

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65
Q

TQ

What is the classification system for NHL? What about staging?

A

R.E.A.L classification

Ann arbor staging like HL

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66
Q

What is more often elevated in NHL, especially indolent lymphomas?

A

Beta-2 microglobulin

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67
Q

What is mandatory for diagnosis of lymphoma?

A

LN biopsy!

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68
Q
  • What is the potentially curative modality used for disease confined to one or two nodal sites on one side of the diaphragm (Stages I and II)
  • Also of value for local compressive symptoms (cord compression, SVC syndrome, tracheal compression from mediastinal mass), and for consolidative treatment of masses treated by chemotherapy
A

Radiotherapy

If high risk (large or 3+ on one side of diaphragm) then give chemo first

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69
Q

How do we treat stage III and IV NHL? What is the combination?

A
  • Chemo

- RCHOP (Rituximab (CD20), Cyclophosphamide, doxorubicin, vincristine, prednisone)

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70
Q

What is the treatment of aggressive lymphoma?

A
  • combination chemo at ALL STAGES

- radiation to shrink large masses

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71
Q

What is the treatment of highly aggressive lymphoma?

A

-Treat like acute lymphocytic leukemia

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72
Q

Prognosis:

  • Indolent: median duration of survival is _ years (most incurable)
  • Aggressive: median duration of survival is _ years, but the 50% of patients who make it past 5 years are cured
  • Patients who are treated at a lower stage have better prognoses than those patients with the same histology who are treated at a higher stage
A

8

4

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73
Q
  • Disorders of the pluripotential stem cell
  • Characterized by ineffective hematopoiesis (Proliferative defects, differentiation abnormalities, impaired apoptosis)
  • Pancytopenia with hyperplastic marrow
  • Usually occurs 60-70 yo
A

Myelodysplastic syndromes

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74
Q

Causes of myelodysplastic syndromes?

A
  • Chemo for other cancers (breast, HL, NHL: alkylating agents and anthracyclines)
  • Radiation (atomic bomb survivors, Chernobyl, therapeutic)
  • Petrochemical exposure (farmers)
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75
Q

TQ!

What are the cytogenetic abnormalities assoc with myelodysplastic syndromes?

A
  • Partial or total loss of long arm of chromosome 5 or 7
  • Inversion of chromosome 16
  • Trisomy 8
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76
Q

Pt presents with…

  • Constitutional symptoms: Fatigue, pallor, bleeding, infection
  • Pancytopenia
  • Incr LDH
  • Incr serum ferritin
  • serum Fe and TIBC normal
A

Myelodysplastic syndrome

half are asymptomatic

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77
Q
  • Erythropoiesis varies from hypoplasia to hyperplasia, often massively ineffective
  • Other cell lines involved (WBCs, platelets) and may have cytopenias in peripheral blood with decreased or increased activity in marrow
  • Often megaloblastic (distinguish from B12/folate)
  • Often asymptomatic but some require transfusion or supp EPO
A

Refractory Anemia (RA)

Subsets: Refractory cytopenia with uni or multi lineage dysplasia

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78
Q
  • Ringed sideroblasts in marrow precursors
  • Mitochondria laden with Fe encircling the nucleus of the erythroid precursors
  • Lowest risk of conversion to AML (10-15%)
A

Refractory Anemia with Ringed Sideroblasts (RARS)

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79
Q

TQ

What must we check in RARS pts to be sure of our diagnosis?

A

Check B6 level on every patient with ringed sideroblasts!

  • Pyridoxine Deficiency (Vit B6 def) also causes ringed sideroblasts
  • Replace B6 for at least 6 months if deficient, if no improvement=pyridoxine resistant sideroblastic anemia (RARS)
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80
Q
  • Refractory anemia with 5-20% myeloblasts in bone marrow
  • High rate of conversion to AML (50%)
  • Often profound pancytopenia
  • Most pts transfusion dependent
  • Cytogenetic abnormalities more pronounced that RA or RARS
A

Refractory Anemia with Excess Blasts (RAEB)

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81
Q

TQ

  • Refractory anemia with 20-30% myeloblasts in bone marrow
  • Cytogenetics even more abnormal
  • ***Highest rate of conversion to AML (65-75%)
  • Circulating myeloblasts in peripheral blood in 25-40% of cases
  • Often managed like de novo AML but much more refractory to AML-style treatment
A

Refractory Anemia with Excess Blasts in Transformation (RAEB-T)

(to AML…)

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82
Q
  • Trilineage dysplastic changes in hematopoietic precursors separate this as dysplasic rather than proliferative
  • Marrow myeloblasts 5-30%
  • Absolute monocyte count > 1000/cc OR > 10% monocytoid cells in marrow
  • Very chronic clinical course with ~25% conversion rate to AML
  • NO Philadelphia chromosome!
A

CMML

NOT a variant of CML

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83
Q 
Myelodysplasias clinical course:
-poor prognosis: \_\_ yr survival
Adverse prognosis:
-Marrow blasts > 5% 
-Platelets  60 years
A

2

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84
Q

Cytogenetic abnormalities–>prognosis in myelodysplasias?

A
  • Monosomy 7
  • Hypodiploidy
  • Multiple abnormalities
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85
Q

TQ!!

Favorable prognosis in myelodysplasias? (cytogenetic)

A

5q- syndrome: beneficial responses to lenalidomide reported

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86
Q

TQ

How do we score myelodysplasia?

A

IPSS score! 4 risk groups..
Low: IPSS 0, survival 5.7 yrs
Intermed 1: IPSS 0.5-1.0, survival 3.5 yrs
Intermed 2: IPSS 1.5-2.0, survival 1.2 yrs
High: IPSS 2.5-3.5, survival 0.4 yrs (4 months)

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87
Q

Supportive Care for myelodysplasia:
-Avoid medications that damage marrow
-Aggressive treatment of infections
-Transfuse PRBCs when symptomatic
-Transfuse platelets only for bleeding or in preparation for surgery
-Watch for iron overload-desferrioxamine (Desferal) if present
-Supplemental vitamins not needed if chemical assays normal
(B6 may be exception)
-Androgens effective if hypoplastic marrow (lots of SE)
-NO Corticosteroids (toxicity)

What is the importance of keeping EPO below 500?

A

Erythropoietin may decrease or ameliorate transfusion requirement in some pts! However, serum EPO level > 500 predicts for poor response

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88
Q

TQ!
Pt <60 yo in good status with IPSS intermed 2 or high risk myelodysplasia categories are considered for what type of therapy?

What if they were IPSS low or intermed-1 category?

A

High intensity therapies

Low intensity therapy/supportive care

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89
Q

TQ
Pt >60 yo in good status with IPSS intermed 2 or high risk myelodysplasia categories are considered for what type of therapy?

What if they were IPSS low or intermed-1 category?

A

Low intensity therapies b/c of age! (selective for high intensity depending on health)

Low intensity therapy/supportive care

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90
Q

TQ

T/F: Myelodysplasia pts with poor performance status receive palliative care regardless of age

A

TRUE

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91
Q

TQ
Low/intermediate intensity therapy for myelodysplasia consists of what two drugs?

Which third drug is used ONLY in 5q- syndrome?

A

Hypo/demethylating agents:

  1. Azacitidine (Vidaza®)
  2. Decitabine (Dacogen®)
  3. Lenalidomide (Revlimid®)
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92
Q

High intensity therapy for myelodysplasia includes what type of therapy?

A
  • AML induction-style treatment
  • Not as effective as de novo AML

(-Response rate=54% with 15% mortality rate at 30 days
-Medial survival 13-15 months)

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93
Q

When should hematopoietic stem cell transplantation be considered in myelodysplasia?

A

-Pts <60 w/ HLA-matched sibling donor

(Significant chance of cure after allo-HCT in low and intermediate risk patients, high transplant-related mortality and relapse)

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94
Q

All of the following are examples of what type of syndromes?

  • Polycythemia Vera (P.V.)
  • Essential Thrombocythemia -Chronic Myelocytic Leukemia (CML)
  • Myelofibrosis (Agnogenic Myeloid Metaplasia, AMM)
  • Sometimes Chronic Lymphocytic Leukemia (CLL)
A

Myeloproliferative Syndromes

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95
Q
  • Increased #s in the peripheral blood of any of the circulating cellular elements,
  • Result from primary proliferation of stem cells in the marrow
  • Chromosomal aberrations include JAK-2 and MPL (thrombopoietin receptor)
A

Myeloproliferative Syndromes

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96
Q
  • Panhyperplasia of marrow (panmyelosis)
  • Involves all cell lines (esp erythroid precursors)
  • A clonal stem cell disorder
  • Male:Female Ratio = 1.4:1
  • Higher incidence in Jews of European extraction
  • Rarely familial
  • 80% of all cases occur in patients over age 50
  • Chronic malignancy
A

Polycythemia Vera

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97
Q 
TQ!!
Pt presents with...Dx?
-**Facial rubor (red face)
-**Pruritis with hot shower or bath
-Hyperviscosity signs such as headache, dizziness, blurred vision, heaviness in arms or legs
-Splenomegaly
A

Polycythemia Vera

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98
Q

What lab findings will you see in PV?

A

-Incr RBC numbers (Hb/Hct)
-Incr LAP
-Incr WBC and/or platelet counts
(20-50,000/μl WBC & 650,000-1,000,000/μl platelets)

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99
Q

TQ

How does PV BM histo differ from myelodysplasia?

A

Hypercellular but NO dyserythropoiesis!

Also see incr reticulin later on (spent phase)

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100
Q

TQ!!

  • Without treatment, 50% mortality at 18 months
  • With treatment patients can live for years to decades
  • Usual cause of death in treated patients is progressive marrow fibrosis with pancytopenia a.k.a. “spent phase” polycythemia

What is the MOST important risk assoc with PV?????

A

Since incr risk of thrombotic and hemorrhagic complications PV has been linked to Budd-Chiari Syndrome!!!!

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101
Q

Why are the following all impt when diagnosing a pt with PV?

  • Hemoconcentration (dehydrated)
  • Pulmonary disease-COPD (smokers polycythemia)
  • EPO producing tumors (renal cell carcinoma, neuroendocrine tumors)
  • Hemoglobinopathy with high affinity hemoglobin
  • Living at high altitude
A

These are all differentials and secondary causes of elevated RBCs…must exclude each one by one when working up for PV!

(Eval lung with pulse oximetry and PFT w/ DLCO)

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102
Q

What mutation is present in over 95% of patients with myeloproliferative disorders including PV?

A

Janus Kinase (JAK) Mutations (JAK2/V617F)

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103
Q

How do we tx PV?

Goal=lowering of RBC mass essential to avoid hyperviscosity complications

A

Phlebotomy!

250-500 cc whole blood every 1-2 weeks as long as Hct > 50%

Avoid alkylating agents (cause leukemia)

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104
Q

A primary myeloproliferative disorder characterized by marrow fibrosis and extramedullary hematopoiesis

A

Myelofibrosis

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105
Q

What is the Myelofibrosis triad?

A

Leukoerythroblastic anemia
Poikilocytosis
Splenomegaly

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106
Q
  • Increased reticulin deposition in marrow
  • Secondary to incr platelet derived growth factor (PDGF) and other cytokines in marrow
  • Increased marrow megakaryocytes can be seen
  • Chronic stimulus to marrow fibroblasts that then make reticulin
  • Marrow architecture disrupted with subsequent mobilization of marrow stem cells to extramedullary sites (spleen, liver, lungs)
A

Myelofibrosis

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107
Q

T/F

JAK2 mutations are always seen in myelofibrosis ?

A

FALSE!

Can assist in diagnosis, but a large percentage of true primary myelofibrosis patients can be JAK negative

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108
Q

Myelofibrosis:
-Chronic, asymptomatic, and progressive with pancytopenia and organomegaly later

How do we treat?

A
  • Observe! No therapy available for reversal of fibrosis
  • Manage pancytopenia with transfusion or EPO
  • Hydroxyurea helps with splenomegaly

ONLY cure is stem cell transplant

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109
Q

What is the new JAK inhibitor approved for treatment of intermediate and high risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis?

A

Ruxolitinib (Jakafi®)

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110
Q

Primary marrow disorder characterized by increased platelet numbers in peripheral blood and increased megakaryocytes in marrow

What is this disorder called and what is the main assoc. problems?

A

Essential thrombocythemia

  • Resultant problems are thrombosis and bleeding
  • Note: increased risk of development of leukemia
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111
Q

What is the criteria for dx of ET?

A
  • **Sustained platelet count ≥450,000/cc
  • Bone marrow biopsy: prolif of the megakaryocytic lineage with increased numbers of enlarged, mature megakaryocytes
  • no significant increase or left-shift of neutrophil granulopoiesis or erythropoiesis
  • Not meeting WHO criteria for PV, MF, CML, MDS or other myeloid neoplasm
  • Demonstration of JAK2 or other clonal marker, or in the absence of a clonal marker, no evidence for reactive thrombocytosis
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112
Q

TQ

What are the 2 main thrombotic complications of ET?

A
  • Digital ischemia
  • Erythromelalgia

(others include:TIA/Stroke, amaurosis fugax, MI, migraine, syncope, Budd-Chiari syndrome)

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113
Q

TQ

  • Burning pain accompanied by redness and increased skin temperature
  • Exacerbation of symptoms with increasing temperatures or cooling the skin
  • Biopsy demonstrates arteriolar inflammation and thrombotic occlusions
  • ASA improves symptoms
A

Erythromelalgia assoc with ET

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114
Q

Pt presents with complaints of incr bruising, epistaxis, superficial mucosal hemorrhages, and bleeding post surgery

Clinical exam otherwise normal

A

Consider ET

115
Q

Lab findings for ET?

A
  • Incr platelet count (even > 1,000,000/μl)
  • Normal or incr WBC count
  • Normal or decr Hb/Hct
  • Marrow exam…normocellular to hypercellular w/ incr megakaryocytes and minimal reticulin
  • Abnormal platelet fx despite normal bleeding time
116
Q

TQ

What disease is ET assoc with?

A

von Willebrand’s disease (vWD)
(This is an acquired form of vWD, NOT a familial disorder!)

  • Reduction in large multimer forms of vWF protein
  • Resembles Type II vWD
117
Q

TQ

Tx of ET if asymptomatic?

A

NONE! Dont tx!!

If symptomatic, hydroxyurea, busulfan, chlorambucil, melphalan all appear to be effective with plateletpheresis or alfa IFN

118
Q

A new agent with antiproliferative properties, helpful in treating ET if refractory to other meds

A

Anagrelide (Agrylin®)

119
Q

Are JAK inhibitors approved for ET tx?

A

NO!

JAK inhibitors have NOT been approved for treatment of ET!

120
Q

All of the following are examples of what type of disease?

  • Monoclonal Gammopathy of Unknown Significance (MGUS)
  • Solitary Plasmacytoma of Bone
  • Indolent myeloma
  • Multiple myeloma
  • Waldenström’s macroglobulinemia
  • Heavy Chain Diseases
A

Specific Plasma Cell Dyscrasias

121
Q

Plasma cell dyscrasia:

  • A condition in which there is clonal proliferation and immortalization of immunoglobulin-secreting plasma cells
  • Immunoglobulin gene rearrangements lead to expression of a unique idiotype

What is the hallmark feature?

A
  • Isolated peak in the gamma region on serum protein electrophoresis=immunoglobulin molecule (the myeloma protein, or M-protein)
  • In some diseases, only light chains or heavy chains may be produced
122
Q
  • Presence of M-protein in serum or urine without identifiable disease
  • M-protein < 3 gm/dl for IgG or < 2.5 gm/dl for IgA
  • Urine light chains < 5%
A

Monoclonal Gammopathy of Unknown Significance

123
Q

Which Ig do most MGUS pts secrete

A

IgG!

124
Q

MGUS is in 1% of pts >50 and in 3% of pts >70 and is usually asymptomatic

So why do we worry about it?

A
  • Immune hemolysis, peripheral neuropathy, renal disease may occur
  • 20% develop B cell neoplasia, myeloma, macroglobulinemia, or lymphoma
125
Q

How do we tx MGUS?

A
  • Supportive care only (unless malignancy is present)

- May need to treat associated problems (plasmapheresis?)

126
Q
  • Single area of bone destruction
  • Negative bone survey and MRI of thoracolumbar spine
  • Normal bone marrow
  • No or low level M-protein with normal levels of immunoglobulins
  • Local radiotherapy alone (4000 cGy) curative in ~30%
  • Long survival time, median 10 years
A

Solitary Plasmacytoma of Bone

127
Q

-Asymptomatic with coincidental diagnosis of low tumor mass
-Follow on no treatment
-Early progression in some (those with bone lesions, local tumors on MRI of spine, Bence Jones proteins)
-Late progression in most
-All patients retain sensitivity to chemotherapy, thus don’t treat until symptoms of myeloma present
-Long survival time, median 8 years


A

Indolent Myeloma

128
Q
  • A disseminated malignancy resulting from a clonal proliferation of transformed plasma cells in bone marrow
  • Malignant cells secrete M-protein in direct proportion to tumor burden
  • 60 yo, males, blacks
A

Multiple Myeloma

129
Q

Pathophys of Multiple Myeloma?

A

Single plasma cell undergoes malignant transformation–>
Grows–>
Disseminates to distant sites in BM–>
Diffuse nodules w/ sheets of plasma cells within the marrow–>
Release osteoclast activators

130
Q

What kind of anemia does multiple myeloma cause?

A

Normochromic normocytic anemia

  • Inhibition of erythropoiesis
  • Disturbance of marrow architecture
  • Immune mediated hemolysis
131
Q

TQ

What are the main clinical manifestations of multiple myeloma?

A
  • Lytic bone lesions (fracture + bone pain)

- Hypercalcemia with hypercalciuria (STONES)

132
Q

TQ!

Mild hypercalcemia + kidney stones=????

A

workup for multiple myeloma!!!

133
Q

T/F: Multiple myeloma pts have no incr risk of serious systemic infx

A

FALSE!
Over 75% of myeloma patients have a serious systemic infection at some point in disease course due to the following:
-Hypogammaglobulinemia—decr Ig -Increased T-suppressor levels
-Poor antibody response to polysaccharide antigens (bacterial cell walls), despite normal T-cell function

134
Q

TQ

What are the two most frequent infections of multiple myeloma and their pathogens?

A

-pneumonias and pyelonephritis

  • Most frequent pathogens are S. pneumoniae, S. aureus, and K. pneumoniae in the lung &
  • E. coli and other gram negative enterics in the urinary tract
135
Q

What do we see in urine of a multiple myeloma pt? Explain

A

Bence jones proteins
-Light chains are rapidly removed and metabolized by the kidney and appear in urine when renal threshold is excreted (Bence Jones proteins)

(Light chains may also be deposited in tissues (amyloidosis))

136
Q
  • May cause hyperviscosity in a multiple myeloma pt
  • May bind with coagulation factors causing coagulopathy
  • May form cryoglobulin leading to Raynaud’s phenomenon and potential gangrene after exposure to cold
A

M-protein of multiple myeloma

137
Q

What is the most common cause of renal failure in multiple myeloma pts?

  • “Blocked pipe” appearance is hallmark
  • Myeloma kidney—eosinophilic casts surrounded by epithelial syncytium in distal tubules and collecting ducts
  • Acquired Fanconi’s syndrome may be seen
A

calcium nephropathy

138
Q

TQ!

Along with Rouleaux formation, what is another key feature of multiple myeloma labs?

A

High erythrocyte sedimentation rate (ESR)! (incr protein)

139
Q

What is the classic triad for multiple myeloma?

A
  • Marrow plasmacytosis (> 10%)
  • Lytic bone lesions
  • Serum and/or urine M-protein

(Diagnosis may be made in absence of bone lesions if plasmacytosis is assoc w/ increase in M-protein over time or if extramedullary mass lesions develop (chloroma))

140
Q

Tests ordered for multiple myeloma:

  • CBC
  • Renal function tests
  • Serum calcium and albumin
  • Serum protein electrophoresis (SPEP) OR
  • 24 hour urine protein electrophoresis (UPEP)
  • Bone marrow aspirate

TQ!
How do we image the bones???

A

Skeletal x-ray survey

NOT bone scan!!! not helpful as osteoblast inhibition by OAF’s inhibit uptake of radionuclide


141
Q

TQ

How can we distinguish MGUS from myeloma on electrophoresis?

A

albumin peak smaller than gamma in myeloma and gamma is very high!

In MGUS, the albumin peak is high and the gamma is lower

142
Q

TQ!!

Impt to distinguish situations needing chemo…DO NOT TREAT which pts?!

A
  • Indolent myeloma—can be observed off therapy until symptoms arise
  • MGUS—it’s not a malignancy
  • Solitary plasmacytoma of bone—radiotherapy!
  • Concomitant medical illness making chemotherapy risky (sepsis). Can proceed when stable
143
Q

What is the standard frontline tx for myeloma?

A

Oral angiogenesis inhibitor:

  • Thalidomide (Thalomid®) or
  • Lenalidomide (Revlimid®)..risk in pregnancy
144
Q

Myeloma pt presents with…suspect what?

  • Suspect in patients presenting with lethargy, polyuria, constipation, nausea/ vomiting, mental confusion, or developing coma
  • Degree of symptoms usually correlates better with serum ionized Ca than total Ca
  • Immobilized patients tend to develop hypercalcemia easier than active patients
A

hypercalcemia

145
Q

Tx of hypercalcemia assoc with myeloma?

A
  • Modest increases in serum Ca (11.5-12.0 mg/dl) usually respond to hydration
  • High Ca levels (>16 mg/dl) =emergency: Manage with IV hydration + bisphosphonates (zolendronic acid or pamidronate)

(Parenteral calcitonin may be used if Ca level is very high (>18 mg/dl)…only lasts a few hours)

146
Q

Myeloma pt developing neurologic symptoms in lower extremities plus focal back pain…
Dx?

Standard imaging of choice=MRI

A

Extradural cord compression until proven otherwise!

Spinal cord compression assoc. with myeloma:

147
Q

Tx of spinal cord compression assoc. with myeloma?

A
  • High dose steroids (Dexamethasone 10-30 mg IV bolus, followed by 4-8 mg every 4-6 hours)
  • Emergency radiotherapy (delay in tx=paralyzed)
148
Q

TQ
Tx of lytic bone dz assoc with myeloma? (2)

Must decr risk of fracture!

A 
Pamidronate (Aredia®) 
Zolendronic acid (Zometa®)
149
Q
  • Plasma cell malignancy in which affected cells secrete IgM (high M-protein >3 g/dl)
  • In contrast to myeloma, lymphadenopathy and hepatosplenomegaly
  • **Major feature is hyperviscosity syndrome (–>retinal change)
  • More common in men and >40 yo
  • Minimal excretion in the urine
A

Waldenström’s Macroglobulinemia

150
Q
  • Weakness, fatigue, recurrent infections
  • Epistaxis, visual disturbances, neurologic symptoms (peripheral neuropathy, dizziness, headache, transient paresis)
  • Hepatosplenomegaly, adenopathy
  • **Segmental dilatation of retinal veins (sausage links) on opthalmoscopic exam—characteristic of hyperviscosity states
A

Waldenström’s Macroglobulinemia

151
Q

What kind of anemia is Waldenström’s Macroglobulinemia?

A

Normochromic normocytic anemia:

  • Rouleaux formation and + Coombs test
  • Malignant cells in peripheral blood
  • Cryoglobulinemia—Raynaud’s phenomenon and cryopathic changes may occur
152
Q

Tx of Waldenström’s Macroglobulinemia?

A

Plasmapheresis can decrease M-protein levels quickly since

Identical tx to that of myeloma

Median survival is > 3 years

153
Q

TQ!!!
-Rare lymphoplasmacytic malignancies in which a defective heavy chain is secreted

  • Heavy chain usually has intact Fc fragment
  • Gamma, alpha, delta, and mu diseases have been reported
A

Heavy Chain Disease

154
Q

TQ
-a.k.a. Franklin’s Disease
-lymphadenopathy, fever, anemia, malaise, hepatosplenomegaly, and weakness
-Palatal edema—nodal involvement of Waldeyer’s ring–>obstructive respiratory symptoms
-Disease course—rapid deterioration and death from infection


A

Gamma Heavy Chain Disease

155
Q

TQ

Dx of Gamma Heavy Chain Disease?

A

Serum M-protein that reacts with anti-IgG reagents but not anti-light chain reagents

156
Q

TQ

  • a.k.a. Seligmann’s Disease
  • Most common heavy chain disease
  • Related to Mediterranean lymphoma (immunoproliferative small intestinal disease —IPSID)
  • Known relationship to intestinal parasites
  • Infiltration of lamina propria of intestine with lymphoplasmacytic cells that secrete truncated alpha chains
A

Alpha Heavy Chain Disease

157
Q

TQ

  • Chronic diarrhea
  • Weight loss
  • Malabsorption
  • Mesenteric and paraaortic adenopathy
  • Respiratory and upper aerodigestive involvement rarely

Which heavy chain dz and treatment?

A

Alpha Heavy Chain Disease

  • Chemotherapy may produce long-term remissions
  • Some patients respond to antibiotics—possible chronic antigenic stimulation by intestinal pathogen is consideration as etiology
158
Q

-Occurs in very rare subset of patients with chronic lymphocytic leukemia
-May see vacuoles in malignant lymphocytes and excretion of kappa light chains in urine
-Tumor cells seem to have defect in secretion of BOTH light and heavy chains
-Treatment is per typical chronic lymphocytic leukemia


A

Mu Heavy Chain Disease

159
Q
  • Systemic illness resulting from deposition of polymerized immunoglobulin light chain fragments in organs and tissues
  • Ig molecules are arranged in beta-pleated sheet configuration
  • Appear as homogeneous pink-staining material on light microscopy of H & E stained tissue sections
A

Primary Amyloidosis

160
Q

Clinical features of _____ ________:

  • Musculoskeletal—decreased joint range of motion, carpal tunnel syndrome
  • Neurologic—peripheral neuropathy, postural hypotension
  • Hematologic—easy bruising and bleeding (endothelial damage)
A

primary amyloidosis

161
Q
  • Edema
  • Weakness, light headedness
  • Paresthesias
  • Shortness of breath
  • “Shoulder pad sign”—from shoulder muscle infiltration
  • “Raccoon sign”—from periorbital hemorrhage
A

Primary amyloidosis

162
Q

Leading cause of death of primary amyloidosis?

A

-Renal failure and heart failure leading causes of death

  • Median survival one year
  • Treating underlying disorder early in its course may be helpful, but of little benefit for advanced cases
  • Amyloid rarely regresses once deposited
163
Q
  • Arises from epidermal keratinocytes
  • Usually seen in fair-skinned persons with excessive sun exposure
  • MC in men (3:1)
  • Typical risk factors, including HPV infection
A

Squamous cell carcinoma

164
Q
  • Sun-exposed areas
  • Red, hardened papule
  • Actinic keratosis
  • Occurs in older pts

Clinical presentation of:

A

Squamous cell carcinoma

165
Q

Precursor lesions of squamous cell carcinoma:

A
  • Bowen’s dz (carcinoma in situ)
  • Cutaneous horn
  • Chronic ulcers
  • Scar tissues
  • Radiodermatitis
166
Q

How is SCC diagnosed?

A

Biopsy – punch, shave, excision

167
Q

Treatments for SCC:

A
  • Cryosurgery
  • Surgical excision
  • Topical chemotherapy (5-FU)
  • Systemic chemotherapy (5-FU, Cisplatin) if metastatic dz for palliation
168
Q
  • Arises from immature pluripotent cells of the epidermis
  • Accounts for 70% of all non-melanoma skin cancers
  • Sun-exposed areas
  • Assoc with basal cell nevus syndrome, Bazex’s syndrome, and the genodermatoses
A

Basal cell carcinoma

169
Q
  • Tends to occur in older pts
  • Slowly growing, shiny, pink papule with telangiectasias
  • Over time, ulcerates and forms rolled borders
A

Basal cell carcinoma

170
Q

Treatments for basal cell carcinoma:

A
  • Surgical excision
  • Mohs surgery
  • Electrodissection
  • Cryosurgery
  • Laser treatment
171
Q

Treatments of recurrence of basal cell carcinoma:

A
  • Repeat resection
  • Radiation therapy
  • Topical chemotherapy
172
Q

What are the unique arm presentations for melanoma between men and women?

A

Man’s LEFT arm

Woman’s RIGHT arm

173
Q

Risk factors for melanoma:

A
  • As per non-melanoma skin cancers
  • Dysplastic nevus syndrome
  • Actinic keratoses (topical liquid N2 or 5-FU)
174
Q
  • MC histological variety of melanoma (70%)
  • Generally arises in preexisting nevus
  • Often slowly changes over months to years
  • Characteristic notching or indentation of the perimeter of the lesion
A

Superficial spreading melanoma (SSM)

175
Q
  • 2nd MC histological variety of melanoma (15-30%)
  • More aggressive
  • Blue-black in color with faster growth rate than SSM
  • Often raised or dome-shaped
  • Lack horizontal growth phase, thus have sharply demarcated borders
A

Nodular melanoma

176
Q
  • Uncommon histological variety of melanoma (4-10%)
  • Low potential for metastasis
  • Typically occur on face of older white women (uncommon before age 50)
  • Usually large (> 3cm)
  • Sun related changes in dermis and epidermis needed for diagnosis
  • Borders often convoluted with prominent notching and indentation
A

Lentigo maligna (Hutchinson’s freckle)

177
Q
  • Characteristically appear on palms of hands and soles of feet or on nail beds (subungual melanoma)
  • MC in non-whites
  • Occur in older pts (> 60)
  • May see a “haphazard” array of color
A

Acral lentiginous melanoma

178
Q

Describe melanoma staging in terms of Breslow thickness.
Thin melanomas:
Thick melanomas:

A 
Breslow thickness – measured using ocular staging micrometer.
Thin melanoma (< 0.75mm) have approx 85% DFS at 10 years post-diagnosis.
Thick melanoma (> 4.0mm) have < 20% DFS at 5 years post-diagnosis.
179
Q

Flat lesion with well-circumscribed change in skin color < 1 cm:
e.g., Freckle

A

Macule

180
Q

Macule > 1 cm:

e.g., Large birthmark (congenital nevus)

A

Patch

181
Q

Elevated solid skin lesion < 1 cm:

e.g., Mole (nevus), acne

A

Papule

182
Q

Nonpustular, non-vesicular lesion on skin > 1 cm:

A

Nodule

183
Q

Small fluid-containing blister < 1 cm:

e.g., Chicken pox (varicella), shingles (zoster)

A

Vesicle

184
Q

Large fluid-containing blister > 1 cm:

e.g., Bullous pemphigoid

A

Bulla

185
Q

Vesicle containing pus:

i. e., collection of leukocytes in the epidermis
e. g., Pustular psoriasis

A

Pustule

186
Q

Papule > 1 cm:

e.g., Psoriasis

A

Plaque

187
Q
  • Erythematous lesion
  • Non-scaling
  • Localized with sepsis

What condition meets this description?

A

Cellulitis

188
Q
  • Erythematous lesion
  • Non-scaling
  • Localized without sepsis

What 3 conditions meet this description?

A

Insect bites
Acne
Hemangioma

189
Q
  • Erythematous lesion
  • Non-scaling
  • Generalized +/– systemic signs

What 4 conditions meet this description?

A

Urticaria
Erythema nodosum
Viral exanthems
Annular erythemas

190
Q
  • Erythematous lesion
  • Scaling
  • Papulosquamous

What 4 conditions meet this description?

A

Pityriasis
Psoriasis
Tinea corporis
Syphilis

others…

191
Q
  • Erythematous lesion
  • Scaling
  • Eczematous

What 3 conditions meet this description?

A

Atopic dermatitis
Contact dermatitis
Scabies

others…

192
Q
  • Xerosis (dry skin) and ichthyosis vulgaris (dry, scaly skin)
  • Erythematous (pigmentary changes)
  • Eye and periorbital changes
  • Hand and foot dermatitis
  • T-cell pathogenesis
A

Atopic dermatitis

193
Q
  • Acute, painful, spreading infection of dermis and subcutaneous tissues
  • Tender, warm, poorly demarcated, boggy plaque
  • MC on extremity
  • Often due to trauma or another infection
  • Usually S. pyogenes or S. aureus
A

Cellulitis

194
Q
  • Symmetric, erythematous nodules and plaques
  • Usually on anterior shins *
  • Often idiopathic, but can be assoc w/ sarcoidosis, coccidioides, histoplasmosis, TB, strep infections, leprosy, and Crohn’s
  • Peak age 20-30
  • 1-15 cm in size
  • Rarely ulcerate
A

Erythema nodosum

195
Q
  • Papules and plaques with silvery scaling
  • Common on knees and elbows
  • Koebner phenomenon – lesions develop along site of injury
  • Auspitz sign – pinpoint bleeds when scales are scraped off
  • Woronoff’s ring
  • Acanthosis with parakeratotic scaling (nuclei still in stratum corneum)
  • Club-shaped rete ridges
  • Absent granular layer
  • Assoc w/ nail pitting and psoriatic arthritis
  • HLA-B13
A

Psoriasis

196
Q
  • Subtype of psoriasis
  • Sterile sheets of pustules on an erythematous base
  • Localized or generalized
  • Painful with fever
A

Pustular psoriasis

197
Q
  • Subtype of psoriasis
  • Children and young adults
  • Post-streptococcal infection
  • Trunk with sparing of palms and soles
A

Guttate psoriasis

198
Q
  • MC ages 55-60
  • Local radicular pain 2-3 days prior to eruption
  • Steel-gray nuclei, multinucleated cells with eosinophilic intranuclear inclusions *
A

Herpes zoster (VZV)

199
Q
  • Branny, greasy red scale on scalp / face / chest / penis
  • P. ovale infection
  • Abnormal immune response to P. ovale with normal amt of yeast
  • Free fatty acid release
  • M > F
  • All races, puberty to adulthood
A

Seborrheic dermatitis

200
Q
  • Corynebacterium minutissimum infection (G+ rod) affecting the intertriginous areas (toes/groin/axilla)
  • Red/brown scaly macules with an erosive collarette-like scale
  • WOOD’S LAMP
  • Chronic
  • Assoc w/ diabetes
A

Erythrasma

201
Q
  • Pruritic, planar, polygonal, purple papules and plaques (6P’s) *
  • Often with reticular white lines on their surface (Wickham’s stria)
  • MC on wrists, elbows, and oral cavity (oral = Wickham’s stria)
  • ‘Saw-tooth’ appearance on histo *
  • Assoc w/ chronic hep C *
A

Lichen planus

202
Q
  • Rose or pink-colored scaly patches or thin plaques
  • Increase in CD4 and Langerhans cells in dermis
  • “Herald patch” following days later by “Christmas tree” distribution *
  • Multiple plaques with collarette scale
  • Self-resolving in 6-8 weeks
A

Pityriasis rosea

203
Q
  • Tinea corporis
  • Superficial infection of skin
  • Trichophyton, Microsporum, Epidermophyton
  • Anthropophilic, zoophilic, geophilic
A

Dermatophyte infection

204
Q
  • Reddish-orange scaling plaques of unknown etiology
  • Keratoderma on palms and soles and follicular keratotic papules
  • AD
  • Similar features of vitamin A deficiency
  • 5 subtypes
A

Pityriasis rubra pilaris

205
Q
  • Subtype of pityriasis rubra pilaris
  • Classic adult type (MC)
  • Red-brown plaques with “islands of sparing”
A

Type 1 pityriasis rubra pilaris

“Classic adult”

206
Q
  • Subtype of pityriasis rubra pilaris
  • Adult atypical type
  • Ichthyosiform
A

Type 2 pityriasis rubra pilaris

‘Adult atypical”

207
Q
  • Subtype of pityriasis rubra pilaris
  • Classic juvenile type
  • Similar to type 1, but before age 2
A

Type 3 pityriasis rubra pilaris

“Classic juvenile”

208
Q
  • Subtype of pityriasis rubra pilaris
  • Circumscribed juvenile type
  • Well demarcated follicular hyperkeratosis along the elbows and knees
A

Type 4 pityriasis rubra pilaris

“Circumscribed juvenile”

209
Q
  • Subtype of pityriasis rubra pilaris
  • Atypical juvenile type
  • Prominent follicular hyperkeratosis with scleroderma-type changes on the palms and soles
A

Type 5 pityriasis rubra pilaris

“Atypical juvenile”

210
Q
  • Subtype of pityriasis rubra pilaris
  • HIV type
  • Orange-red plaques with follicular hyperkeratosis and “islands of sparing”
  • Skin, mucous membranes, nails, eyes
A

Type 6 pityriasis rubra pilaris

“HIV type”

211
Q
  • Scaling plaques which mimic eczema
  • Itchy
  • Multiple different shades of red-brown
  • Round, oval annular, or bizarrely shaped
  • Check for LAD
  • Affects the CD4 T helpers
  • Lymph nodes and organs become involved
  • > 50
  • Males
  • Assoc w/ HTLV
A

Cutaneous T-cell lymphoma

212
Q
  • Scarring atrophic photosensitive dermatosis
  • Erythematous papule or plaque with modest amt of scale
  • Hypo- or hyperpigmented
  • Mucosal, palms and soles
  • 1:2 (M:F)
  • Ages 20-40
  • Genetic predisposition
  • Heat shock protein induced by UV light
  • Mostly asymptomatic, mild itching
  • Look for pericarditis, neurologic sx
  • Malignant transformations (SCC) can occur
A

Discoid lupus erythematosus

213
Q

Drug eruption:

Over-dosage of coumadin…

A

Purpura

214
Q

Drug eruption:

Accumulation of silver nitrate

A

Argyria

215
Q

Drug eruption:

Phototoxic…

A

Doxycycline

216
Q

Drug eruption:

Imbalance of normal flora with ATB

A

Candidiasis

217
Q

Drug eruption:

Killing of bacteria or fungus by appropriate agent causes:

A

Jarisch-Herxheimer rxn

218
Q
  • Very superficial skin infection
  • Usually from S. aureus or S. pyogenes
  • Highly contagious
  • Honey-colored crusting *
  • Common on face, but can be anywhere
A

Impetigo

219
Q
  • Exotoxin destroys keratinocyte attachments in the stratum granulosum only (vs. toxic epidermal necrolysis, which destroys at epidermal-dermal junction)
  • Characterized by fever and generalized erythematous rash
  • Sloughing of upper layers of the epidermis that heals completely
  • Seen in newborns and children (nurseries and daycare)
  • (+) Nikolsky sign
A

Staphylococcal scalded skin syndrome

220
Q
  • IgG (IgG1 and IgG4) against desmoglein (desmosomes)
  • Acantholysis&raquo_space; flaccid intraepidermal bullae
  • (+) Nikolsky sign
  • (+) Asboe-Hansen sign (lateral pressure on the bulla will spread bulla the uninvolved skin)
  • Assoc w/ myasthenia gravis and thymoma
A

Pemphigus vulgaris

221
Q
  • Asymptomatic to painful
  • Prodrome = itching/burning
  • Recurrent lesions common
  • “Vesicular or ulcerative on an erythematous base”
  • Oral, genital, body, keratoconjunctival, encephalitis, anal
A

Herpes simplex

222
Q
  • “Oval teardrop on an erythematous base” *
  • “Dew drop on a rose pedal” *
  • Itchy rash
  • Face, trunk
  • 10-21 day incubation
A 
Chicken pox
(HSV-3 – varicella)
223
Q
  • Less severe than pemphigus vulgaris
  • IgG against hemidesmosomes (epidermal BM – “bullow” the epidermis) *
  • TENSE bulla containing eosinophils *
  • Spare oral mucosa *
  • (–) Nikolsky sign
A

Bullous pemphigoid

224
Q
  • Idiopathic photodermatoses from sun exposure (30 min after)
  • Papule, vesicles and plaques
  • 75% American Indian
  • Females (MC)
  • Type IV hypersensitivity (delayed-type)
  • UV-A-induced ICAM-1
  • Spring, lessens throughout summer
A

Polymorphous light eruption

225
Q
  • Pruritic papules, vesicles, and bullae
  • Often found on elbows
  • IgA at tips of dermal papillae *
  • Assoc w/ celiac disease (pts have gluten sensitivity) *
  • HLA-B1, B8, DR8, DRQ
  • Rx: dapsone
A

Dermatitis herpetiformis

226
Q
  • Assoc w/ infections (e.g., Mycoplasma pneumoniae, HSV*), drugs (e.g., sulfa drugs, B-lactams, phenytoin, allopurinol), cancers, and autoimmune dz
  • Mucosal involvement
  • Classically seen as target lesions *
  • Iris-shaped macules or vesicopapules on the palms or soles *
  • 50% under 20
  • Koebner phenomenon (lesions arising on sites of trauma)
A

Erythema multiforme

227
Q
  • “Extensive full thickness skin detachment”
  • Cell-mediated cytotoxic rxn against epidermal cells
  • “sheet-like” epidermal loss *
  • Prodrome = 1-3 weeks
  • > 30% of the body surface area involved (
A

Toxic epidermal necrolysis

228
Q

Risk factors for skin cancer?

A
  • contact carcinogens (bruns, chimney soot)
  • **sunlight (290-320 nm, >3 burns)
  • tanning beds (UV-A)
  • UV light (pyrimidine dimers–>C-T mutation)
  • Genodermatoses (recessive)
229
Q

Women who use tanning beds are ___% more likely to develop skin cancer

A

55

230
Q

Screening for cancer:

  • Importance?
  • ALL skin surfaces
  • Self: Monthly if 30-40yo
  • Physician too! annually
A

self-exam minimizes late-stage findings esp in high risk pts (work outside, genetics)

231
Q 
TQ
Why is it important to recognize skin types?
Ex:
I always burns, never tans
2 always burns, minimal tan
3 Burns often, tans gradually
4 Burns minimally, tans well
5 Burns rarely, tans profusely
6 Never burns, deeply pigmented
A

-Incidence of skin cancer is directly related to potential to burn!

232
Q
  • arises from epidermal keratinocytes
  • usually in fair-skinned ppl w/ excessive sun exposure
  • Men>women
  • HPV incr risk
A

squamous cell carcinoma

233
Q
  • usually on sun exposed areas
  • Red, indurated papule
  • actinic keratosis
  • older pts

What are some precursor lesions of this skin cancer?

A

(squamous cell carcinoma)

  • **Bowen’s dz (in situ)
  • cutaneous horn
  • chronic ulcers
  • scar tissues
  • radiodermatitis
234
Q

How do we dz and treat squamous cell carcinoma?

A
  • biopsy: punch, shave, excision

- tx: cryosurgery, excision, topical chemo (5-FU), systemic chemo (5-FU, cisplastin) if mets

235
Q
  • arises from immature pluripotential cells of epidermis
  • slow growing, shiny, pink papule w/ telangiectasias–>ulcerates w/ rolled borders
  • sun-exposed areas usually
  • older pts
  • 70% of nonmelanomas

what is this carcinoma assoc with?

A

(Basal cell carcinoma)

Assoc with basal cell nevus syn, bazex’s syn, and genodermatoses

236
Q

Tx of basal cell carcinoma?

Biopsy first!

A
  • Excision
  • Mohs sx (mowing lawn…one way then another)
  • Electrodessication: fry off
  • Cryosurgery
  • Laser tx

(If reccurence then repeat or add radiation/chemo)

237
Q
  • avg onset 57 yo but seen in 20-30s
  • male>female
  • less common in blacks, asians, hispanics
  • closer to equator=higher

risk-arm presentations (men’s left, women’s right)

A

melanoma

238
Q

TQ
Dysplastic nervus syndrome is a risk factor for melanoma

What is the inheritance pattern of dysplastic nervus syn??

A

Autosomal dominant!

multiple family members affected

239
Q

Other risk factors for melanoma:

  • _____ ______: scaly, rough, erythematous patches on sun exposed areas
  • May progress to SCC and melanoma
  • Topical 5-FU
A

actinic keratosis

240
Q

What are the four types of melanoma?

A
  • Superficial spreading
  • Nodular
  • Lentigo maligna
  • Acral lentiginous
241
Q
  • MC variety of melanoma
  • Arises in pre-existing nevus
  • Slowly changes
  • Nothing or indentation of perimeter
A

Superficial spreading melanoma

242
Q
  • 2nd MC melanoma
  • More aggressive
  • Blue-black color and faster growth
  • Raised/dome shaped
  • Lack horizontal growth (deep)
A

Nodular melanoma

243
Q
  • Uncommon melanoma
  • Low potential for mets
  • Older white women
  • Large (>cm)
  • Sun related changes
  • Borders convoluted with prominent notching and indentation
A 
Lentigo Maligna (Hutchinson's freckle)
"see on grandma's face"
244
Q

TQ

  • palms of hands, soles of feet
  • **nail beds (subungual melanoma)
  • non-whites!!!
  • older pts
  • disorganized array of color
A

Acral lentiginous (acral means tips/ends)

245
Q

In transit metastasis: nodules representing deposits of melanoma as it spreads to LN

Sign of what?

A

Metastasis or reoccurrence

246
Q

Pt presents with slate gray appearance

A

disseminated melanoma (melanosis)

247
Q

TQ

How do we stage melanoma?

A

-Determine the depth!
(breslow’s thickness)
Entire lesion measured
-thin has much better prognosis than thick:
Thin (4.0 mm) have less than 20% dz free at 5 yrs

248
Q

Melanoma pts are grouped into AJCC stage by ____ status (thickness, LN, mets)

LN status with melanoma:
-Males, over 50 w/ thick melanoma
-ulceration
-dx post biopsy or lymphadenectomy
Mets:
-separation b/t local mets and distant mets (organs)
-mets to lung are a unique subset
CATCH EARLY
A

TNM status

249
Q

TQ

Most important feature of melanoma?

A

Thickness!!

250
Q

Surgery for melanoma:

  • wide local excision + normal tissue
  • may have satellite focus
  • survival correlates with ________
A

thickness

251
Q

Surgery for melanoma:

  • in situ: ____ cm normal skin
  • 4mm thick: __ cm normal skin
  • fingers/toes: amputation
  • sole of foot: wide excision but save weight bearing tissue (heel, ball)
  • ear: wedge or partial amputation (vanGogh)
  • face: 1 cm :)
A
  • in situ: 0.5-1.0 cm normal skin

- 4mm thick: 3.0 cm normal skin

252
Q

What is the only curative modality for nodal melanoma mets?

-risk for mets incr wtih thickness, ulceration, and location (foot, hand, scalp, face)

A

LN dissection

253
Q

T/F All pts with melanoma 1 mm in breslow thickness should undergo sentinel LN biposy and dissection

A

True!

254
Q

T/F

Melanoma can be treated with radiotherapy

A

FALS
Eradioresistant
-use if painful bony mets or brain mets

255
Q

Systemic therapy for melanoma?

  • melanomas >4mm OR
  • in transit mets OR
  • regional LN mets
A

alpha interferon given in high dose then 3x/wk for 1 yr

  • SE significant
  • improves survival by 25%
256
Q

Systemic therapy for melanoma?
-DTIC, Adriamycin, Cisplatin, Vinca alkaloids, taxanes

-Immunotherapy involves what?

A

Alpha IFN
IL-2
Tumor vaccines

257
Q

Metastatic melanoma tx that require patient selection due to SE?
-_________: monoclonal Ab targeting CTLA-4–>
immune reaction against tumor-_________: BRAF kinase inhibitor, cutaneous toxicity

A

Ipilimumab

Vemurafenib

258
Q

TQ
Which metastatic melanoma tx is actually well tolerated???

Which patient population?

A

Dabrafenib…drug inhibit BRAF in pts with V600E mutation of BRAF (fever, hypergly)

259
Q

TQ

SE of BRAF inhibitors used for melanoma is due to what? What are the SE?

A

Activation of MAP kinase pathway: bypasses inhibition of BRAF–>

  • SCC and keratoacanthomas
  • **Cutaneous: rash, photosensitivity, alopecia
  • QT prolongatoin (CYP34A)
260
Q

TQ

  • New tx for melanoma that initially is approved for BRAF mutant melanoma as a MEK inhibitor
  • approved for use in combo with dabrafenib
A

Trametinib

combo of dabrafenib + trametinib way better but may cause fever!

261
Q
  • Primary cutaneous neuroendocrine carcinoma (small blue cells)
  • Arises from cells in basal layer of epidermis and hair follicles
  • Rapidly growing, bluish red
  • highly malignant (mets early)
  • very rare: fair elderly pts, women do better
A

Merkel cell carcinoma

262
Q

Tx of merkel cell carcinoma?

A
  • wide excision and prophylactic LN dissection

- chemo if metspoor prognosis :(

263
Q
  • bone marrow derived dendritic cells in the upper layer of epidermis
  • limited to one organ system (bone 77%)
  • benign but may be aggressive
  • often treated with chemo agents
A

Tumors of langerhans cells

264
Q

TQ

  • Brown to purplish papules (Hashimoto-Pritzker variant rash)
  • Eczemtous rash (looks like candida)
  • Oral lesions
A

Tumors of langerhans cells (LCH)

265
Q

Tx of LCH?

A

Topical therapy if single system

Multisystem=chemo if symptomatic

INCURABLE

266
Q

TQ

  • single or multiple small nodules on face and neck resembling warts
  • Cowden’s syndrome
  • malignant lesions rare (carcinoma form)
  • local resection establishes dx and is curative when malignant
A

Trichilemmoma (benign) tumor of hair follicle

267
Q

-may be single (nonhereditary) or multiple (autosomal dominant
)-appears as skin colored papules on nasolabial folds, eyelids, and central face
-may be confused with BCC
-local resection curative

A

Trichoepithelioma tumor of hair follicle

268
Q
  • tumors arising from the hair matrix
  • single or multiple
  • invade locally but no mets
  • local resection curative
A

Pilomatricoma tumor of the hair follicle

269
Q
  • small, yellowish papules on the vermillion border of the lips or oral mucosa
  • ectopic sebaceous glands
  • incr w/ age
  • Retier’s syndrome assoc
  • not malignant
A

Fox-Fordyce Anomaly
(tumor of sebaceous gland)
(Fordyce’s spots)

270
Q

-occurs MC in the conjunctiva of the upper eyelids in women 60-80 yrs old-asians-slow but aggressive and mets

A

Sebaceous carcinoma

271
Q
  • benign tumor of eccrine glands
  • airse from outer cells of the intraepidermal duct
  • pendunculated and skin colored
  • sole of foot, palm of hand
  • surgical excision
A

Poroma (benign tumor of eccrine glands)

272
Q
  • benign tumor arising from intradermal coiled duct of eccrine gland
  • multiple, firm, smooth, dome-shaped movable pink to red papules
  • Turban
  • female>male
  • rarely–>carcinoma
  • surgical excision
A

Cylindroma (“turbon tumor”) (benign tumor of eccrine glands)

273
Q
  • solitary eccrine gland carcinoma
  • tumor of face, head, hand, or foot
  • mets
  • differentiate from other primary cancers
A

Clear cell carcinoma

274
Q
  • englarging, ulcerated nodule
  • eccrine gland carcinoma
  • highly malignant
  • differentiate from mets from other sites
A

Eccrine Adenocarcinoma

275
Q

TQ

  • low grade lymphoma of T cells in skin
  • shed lymphoma cells into blood (sezary syndrome)
  • CMV IgG serology
  • tx with topical nitrogen mustard, PUVA, electron beam radiology
A

Cutaneous T cell lymphoma
(mycosis fungiodes)
(lymphoreticular tumor)

276
Q
  • benign vascular tumor that mimics the modified smooth muscle cells of glomus body (AV anas in skin)
  • small, blue-red nodule in subcutaneous tissue or subungual region of finger
  • PAIN
A

Glomus tumor

277
Q

TQ

  • malignant tumors of blood or lympatic vessels
  • Assoc with Stewart-Treves Syndrome!!!
A

Angiosarcoma

278
Q

TQ What is Stewart-Treves syndrome?

(assoc with angiosarcoma)

A
  • Cutaneous lymphangiosarcoma arising in chronic lymphadematous extremities
  • seen in breast cancer
279
Q

-benign lesions common in children
-spontaneously regress!!
dont cut-rapid growth

A

Hemangioma

280
Q

Cavernous hemangioma: multiple large dilated vessels with flattened endothelium

Risk of what?

A

DIC

281
Q
  • malignant vascular lesion in skin of lower extremities
  • mediterranean or jewish
  • mets to lung and GI tract
  • local excision and radiation
A

Kaposi’s sarcoma classic type

(African type in Bantu men in Africa, children, aggressive
Immunocompromised type in HIV, organ transplant pts)

282
Q

-MC of all benign neoplasm
s-anywhere where fat is present
-fat cells
-no tx needed

A

lipoma

283
Q
  • malignant tumor arising from fat
  • 55-60 yo-2nd MC soft tissue sarcoma
  • aggressive surgery!!
  • radiation and chemo (mets)
A

Liposarcoma

SBL Final (4 decks)

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