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Clinical Medicine II > Hematology/Oncology > Flashcards

Hematology/Oncology Flashcards

1
Q

What is wrong in hemophilia?

A

Deficiency of factor VIII or IX (intrinsic pathway) -> insufficient thrombin production

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2
Q

What are the types of hemophilia?

A

Hemophilia A - deficiency of factor VIII

Hemophilia B - deficiency of factor IX

Hemophilia C - deficiency of factor XI

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3
Q

Which hemophilias are recessive X-linked disorders?

A

Hemophilia A & B

Hemophilia C is autosomal disorder

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4
Q

What are the symptoms of hemophilia C?

A

Mild spontaneous bleeding only - just need ppx for surgical procedures

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5
Q

What are the different severities of hemophilia?

A

Severe: <1% active factor
Moderate: 1-5% active factor
Mild: 5-40% active factor

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6
Q

Is hemophilia exclusive to males?

A

Nope! Women can have it, too!

  • Lionization of normal X chromosome (inactivation)
  • Turner syndrome (XO)
  • Father w/ hemophilia + mother as carrier
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7
Q

How would a pt with hemophilia present clinically?

A

Family hx
Gingival bleeding
Easy bruising
Frequent epistaxis
Spontaneous bleeding
Excessive bleeding following trauma/surgery
Hemarthrosis = most common site (“target joint”, debilitating arthritis)

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8
Q

What labs would you need to look at for a hemophilia pt?

A
  • Elevated aPTT (can be normal if factor activity >15%)
  • Reduced factor VIII or IX
  • DNA analysis
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9
Q

Treatment for hemophilia

A
  • Prophylactic factor VIII/IX (recombinant has longer half-life than plasma-derived)
  • Prompt tx of hemarthroses may preserve joint
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10
Q

What do you if a hemophilia pt experiences an acute bleeding episode?

A

Raise the factor level to >50

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11
Q

Uh oh, you don’t have any factor VIII or IX available for your hemophilia pt. Now what?

A
  • Prothrombin complex concentrate
  • Recombinant factor VIIa
  • Desmopressin (stimulates VIII & vWF release from storage) → only works for mild hemophilia A
  • Episolon aminocaproic acid (EACA-Amicar) → anti-fibrinolytic agent for emergency; do NOT give if using prothrombin complex (excessive thrombosis)
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12
Q

What are some possible complications to hemophilia treatment therapies?

A

Transfusion transmitted infections (not as common anymore)

Antibodies against VIII/IX (recombinant factors less reactive)

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13
Q

What is the daily requirement of vitamin K?

A

100-200mcg/day

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14
Q

How does the body get vitamin K?

A

Mainly from diet - absorbed in terminal ileum

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15
Q

Which clotting factors are activated by vitamin K? What else?

A

Factors II (prothrombin), VII, IX, X

Protein C & S (natural anticoagulants that prevent excess clotting)

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16
Q

What are some causes of vitamin K deficiency?

A
  • Low dietary intake
  • Abnormal absorption (bile obstruction)
  • Warfarin
  • Newborns are born with low vit. K levels and don’t have gut flora to make their own yet (hemorrhagic disease of newborn)
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17
Q

How do you treat vitamin K deficiency?

A

Vitamin K supplementation (diet or PO/IV)

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18
Q

What is DIC?

A

Consumption of platelets d/t microvascular thrombi formation

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19
Q

What are some common causes of DIC?

A 
Malignancy
Trauma, burns, infections, sepsis
Transfusion
Obstetric - placental abruption, amniotic fluid embolism
Liver disease
Prosthetic devices
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20
Q

What is the pathophysiology behind DIC?

A

Excessive plasmin (degrades fibrin clots) or excessive thrombin formation

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21
Q

How do pts with DIC present?

A
  • Bleeding & ischemia (e.g. cyanosis)
  • Arterial/venous thrombosis → AKI, MI, DVT
  • Purpura fulminans → cutaneous intravascular thrombosis & hemorrhagic infarction of skin; usually d/t sepsis
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22
Q

What is involved in the work-up for DIC?

A
  • History - trauma, malignancy, sepsis
  • Thrombocytopenia, anemia
  • Incr. thrombin production in early phase
  • Decreased fibrinogen
  • AKI, elevated LFTs
  • Prolonged PT, aPTT (deficiencies of clotting factors)
  • Reduced antithrombin, protein C, S
  • Elevated d-dimer (fibrinolysis)
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23
Q

How do you treat DIC?

A

Treat underlying cause!

  • Hemodynamic support - ABC
  • Infusions for platelets, antithrombin
  • FFP for factor/fibrinogen
  • Protein C conc. if low and purpura fulminans
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24
Q

What is polycythemia vera?

A

Chronic acquired myeloproliferative neoplasm causing overproduction of abnormal BM products (esp. RBC) → mutation in JAK2

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25
Q

What are the consequences of polycythemia vera?

A

Hyperviscosity of blood → Increased cardiac workload

Ischemia/thrombosis of organs

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26
Q

What are the types of polycythemia vera?

A

Primary → mutated JAK2 that makes RBC without EPO activation

Secondary → EPO-driven production of RBCs (no JAK2 mutation)

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27
Q

What can cause secondary polycythemia vera?

A
  • Hypoxia (high altitude, smoking, sleep apnea)
  • EPO-secreting tumors (RCC, pheo)
  • Adrenal (adenoma, Cushing’s)
  • Renal (polycystic kidney disease, hydronephrosis)
  • Testosterone
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28
Q

How would a pt with polycythemia vera present?

A
  • Sweating, H/A, tinnitus, blurred vision, red/purplish skin
  • Spleno/hepatomegaly
  • Thrombosis, bleeding
  • Aquagenic pruritus
  • Erythromelalgia → severe burning pain in hands/feet, usually w/ red/bluish skin
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29
Q

Labs related to polycythemia vera

A
  • Elevated Hgb, Hct, WBC, platelets
  • Low serum EPO
  • BM histopathology shows JAK2 mutation
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30
Q

Treatment for polycythemia vera

A
  • Phlebotomy plus low-dose ASA
  • If high risk, non-compliant on phlebotomy, or progressive (splenomegaly, leukocytosis, thrombocytosis) → hydroxyurea +/- interferon
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31
Q

How are platelets formed?

A

Thrombopoiesis in BM from megakaryocytes → shed directly into BM

Each megakaryocyte produces 50,000-10,000 platelets

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32
Q

How long do platelets live? Where do they go to die?

A

5-9 days → Old platelets are destroyed by spleen via phagocytosis & liver via Kupffer cells

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33
Q

What is thrombocytopenia?

A

<150,000/mcL

Note: 2.5% normal pop. has plt count <150,000

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34
Q

What is the normal range for platelet levels?

A

150,000-450,000/mcL

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35
Q

What are the 4 general causes of thrombocytopenia?

A
  • Decreased plt production
  • Increased plt destruction
  • Dilutional thrombocytopenia → big transfusion of pRBC
  • Distributional thrombocytopenia → cirrhosis, portal htn, splenomegaly
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36
Q

What can cause decreased platelet production, leading to thrombocytopenia?

A

Bone marrow suppression/damage

  • Viral infections e.g. rubella, varicella, mumps, parovirus, HepC, HIV, EBV
  • Chemo/radiotherapy to sites of plt production
  • Congenital/acquired BM hypoplasia/aplasia
  • Alcohol toxicity
  • Vit. B12/folic acid deficiency
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37
Q

What can cause increased plt destruction, leading to thrombocytopenia?

A
  • Autoimmune disorders
  • Post-infection e.g. CMV, mono
  • Drugs e.g. heparin, valproic acid, quinine
  • Alloimmune destruction (post-transfusion/transplant, neonatal)
  • Physical destruction (surgery, cardiac mass, large aneurysm)
  • DIC, sepsis
  • TTP
  • HELLP in pregnant w/ eclampsia
  • Hypothermia
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38
Q

Clinical presentation of thrombocytopenia

A

Can be asymptomatic until plt count <100,000/mcL

Bleeding, ecchymosis, purpura, petechiae

Spontaneous bleeding if <20,000/mcL

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39
Q

What’s wrong in von Willebrand disease?

A

Qualitative/quantitative deficiency of vWF, which is required for platelet adhesion → bleeding

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40
Q

What are the types of vWD?

A
  • Type I (most common) → quantitative defect
  • Type II → qualitative defect
  • Type III → complete absence of vWF production → extremely low VIII (vWF protects against degradation), hemophilia A-like
  • Acquired
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41
Q

What are some causes of acquired vWD?

A
  • Cardiac valvulopathies → mechanical shearing of vWF
  • DIC → degradation by plasmin
  • Hypothyroidism → reduced synthesis
  • Autoimmune diseases
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42
Q

What diagnostics are involved with vWD?

A
  • vWF antigen assay → evaluate quantity/quality
  • Factor VIII levels
  • Normal PT but variable prolongation of aPTT (insufficient VIII)
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43
Q

Treatment for vWD

A
  • Desmopressin → increases vWF/VIII release from endothelial cells; used for ppx and tx
  • Transfusion of vWF-containing VIII conc./FFP
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44
Q

What’s wrong in idiopathic thrombocytopenic purpura (ITP)?

A

Thrombocytopenia with normal BM functioning (diagnosis of exclusion)

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45
Q

What is the pathophysiology behind idiopathic thrombocytopenic purpura (ITP)?

A

Autoimmune - antibodies against plt antigens & IgG autoantibodies that damage megakaryocytes

Inadequate production d/t deficiency of TPO

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46
Q

2 types of ITP

A

Acute → <2 months, s/p infection in children

Chronic ITP → >6 months

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47
Q

At what plt count would a pt be at risk for life-threatening bleed?

A

<20,000/mcL

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48
Q

Treatment for ITP

A

High-dose IV glucocorticoids, immunoglobulin, anti-D Ig (less susceptible to opsonization/phagocytosis)

Platelet transfusion if severe hemorrhage

Splenectomy reserved for failed medical therapy

Admission if not yet diagnosed w/ thrombocytopenia (ITP is dx of exclusion)

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49
Q

What’s wrong in thrombotic thrombocytopenic purpura (TTP)?

A

Inhibition/deficiency of ADAMTS13, which cleaves vWF into smaller units → incr. platelet adhesion to large vWF multimers → microthrombi & platelet consumption

Anemia d/t shear stress to passing RBC

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50
Q

Clinical presentation of TTP

A

Initially nonspecific sx’s (malaise, fever, h/a, n/v/d) followed by bleeding sx’s

FATRN:
Fever
Anemia
Thrombocytopenia (bruising, purpura)
Renal failure
Neuro sx's - hallucinations, altered mental status, visual changes
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51
Q

Diagnosis criteria for TTP

A

3 or more FATRN sx’s with schisctocytes

Normal PTT

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52
Q

Tx of TTP

A

Early plasmapheresis/plasma exchange → reduce ADAMTS13 antibodies & replenishes levels → repeat daily

Refractory/relapsing TTP = additional immunosuppressive therapy

Monitor LDH, plts, schistocytes for disease progression/remission

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53
Q

Cells that arise from lymphoid progenitor

A

NK cells
T cells
B cells

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54
Q

Cells that arise from myeloid progenitor

A

RBC
WBC
platelets

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55
Q

Normal WBC range

A

4,000-11,000

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56
Q

Leukopenia

A

<4,000 WBC

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57
Q

Leukocytosis

A

> 11,000 WBC

58
Q

Normal WBC differential (% of each cell type)

A 
Neutrophils 50-70%
Lymphocytes 20-40%
Monocytes 5-10%
Eosinophils 1-3%
Basophils 0-1%
59
Q

Categorization of hematologic malignancies

A

Acute vs. chronic

Lymphoid vs. myeloid

60
Q

What are lymphomas anyway?

A

Discrete, well-defined mass in bone marrow/lymphoid tissue formed by hematopoietic precursor cells (blasts)

61
Q

Classic symptoms of any lymphoma

A

PAINLESS LYMPHADENOPATHY
Fever of unknown origin
Weight loss

62
Q

Acute vs chronic leukemias

A

Acute → immature-looking cells that behave aggressively & affect normal hematopoietic cell production

Chronic → overload of “mature”-looking cells but do not lead to severe sx’s

63
Q

Epidemiology of AML

A

More common in adults

64
Q

Etiology of AML

A
  • Genetic abnormalities → trisomy 21, Fanconi anemia
  • Radiation → 5-7 yrs after high-dose exposure
  • Chemicals → benzene, smoking, petroleum, paint, embalming fluids, herbicides, pesticides
  • Drugs → 4-6 yrs after alkylating/chemo agents
65
Q

Clinical presentation of AML

A

Non-specific but ABRUPT-ONSET (fatigue, anemia, anorexia, weight loss, bleeding, etc.)

Splenomegaly, LAN, sternal tenderness, gum hypertrophy, papilledema

66
Q

Work-up findings for AML

A

BONE MARROW ASPIRATE & BIOPSY → >20% blasts

Auer rods (M2, M3, M4)

Leukocytosis, blastemia, thrombocytopenia, anemia

67
Q

Treatment for AML

A

Immediate inpatient chemo

SCT if relapse or recurrence

68
Q

3 Phases of CML

A

Chronic (<10% blasts) → asymptomatic, most are diagnosed at this stage

Accelerated (≥15% blasts)

Blast → ≥30% blasts + extramedullary disease → poor prognosis

69
Q

Clinical presentation of CML

A

Lymphadenopathy
Mild hepatomegaly
Splenomegaly (in blast crisis)
Myeloid sarcoma

70
Q

Work-up findings of CML

A

BONE MARROW ASPIRATE & BIOPSY

Cytogenics → Ph chromosome

FISH → bcr/abl arrangement

CBC w/ diff → incr. immature/mature granulocytes (neutrophils, basophils, eosinophils), variable platelets

71
Q

What’s the big deal about Philadelphia chromosome?

A

Abl gene from chrom. 9 inserts into bcr gene in chrom. 22 → bcr/abl oncogene

Associated with CML

72
Q

Tx of CML

A

Imatinib (tyrosine-kinase inhibitor) → inhibits ATP-binding site of bcr-abl oncoprotein → apoptosis

If pt is in accelerated or blast phase, can use imatinib + chemo + consider SCT

73
Q

Indicators of poor prognosis of CML

A
  • Progression to blast phase
  • Older age
  • Severe anemia
  • Negative Ph chromosome
  • Thrombocytopenia
  • Hepatomegaly
  • Splenomegaly
74
Q

Epidemiology of ALL

A

Bimodal distribution

Most common childhood cancer

75
Q

Risk factors for ALL

A
  • Previous cancer tx
  • Exposure to radiation
  • Genetic disorders (Down syndrome, Klinefelter)
  • Sibling w/ ALL
  • Varicella in childhod ALL
76
Q

Clinical presentation for ALL

A

Bone marrow failure → malaise, fatigue, bleeding, bruising, secondary infections

B symptoms → fever, weight loss, night sweats

CNS INVOLVEMENT → cranial neuropathies, meningeal infiltration

77
Q

Work-up for ALL

A

BONE MARROW ASPIRATE & BIOPSY

LP to assess CNS involvement

CBC w/ diff → lymphoblasts, normal/decr. WBCs, anemia, thrombocytopenia

78
Q

Main tx for ALL

A

Chemo w/ CNS prophylaxis

79
Q

What are the 4 components of chemo for ALL tx?

A

Induction → eradication of all detectable leukemia cells (<5% blasts)

Consolidation → minimize risk of relapse

Maintenance → maintain remission

CNS prophylaxis → minimize risk of spread to CNS

80
Q

What happens in induction phase for ALL tx?

A

Eradication of all detectable leukemia cells

4-6 weeks of hospitalized chemo → 3-4 drugs for peds, 4-5 drugs for adults

Add imatinib if +Ph chromosome

Add rituximab if B cells are +CD20

81
Q

What happens in consolidation phase for ALL tx?

A

Minimize relapse risk; pt must be in complete remission (repeat BM biopsy)

Hospitalized chemo → 1-2 months for peds, 6-12 months for adults

Includes some agents from induction phase

82
Q

What happens in maintenance phase of ALL tx?

A

Maintain remission

Lower doses of chemo for 18-24 months

T-cell and mature B-cell ALL may not need maintenance

83
Q

What happens in CNS ppx phase of ALL tx?

A

Begins during induction phase and continues through entire tx regimen

Intrathecal chemo via LP or Onmaya reservoir (intraventricular)

84
Q

What additional tx options can be added onto chemo for ALL tx?

A

Infectious ppx during chemo

Blood bank support → pRBC when Hb <8 or sx’s with comorbidity, platelets when plt<10,000 or <50,000 if active bleed

Neupogen for neutropenia

CAR-T cell tx - remove T cell via apheresis, reengineer in lab and return to body to attack targeted tumor cells

85
Q

Indicators of good prognosis for ALL

A 
<50,000 WBC
Age 1-10
Female
B-cell blasts
Huge reduction of blasts at day 7 of induction
86
Q

Indicators of poor prognosis for ALL

A 
>50,000 WBC
<1 or >10 y/o
Male
T-cell blasts
Mild reduction of blasts at day 7 of induction
87
Q

Markers involved with CLL

A

Expansion of CD5+ B cells

CD38 associated with UNmutated Ig heavy-chain variable gene (IGHV) → worse prognosis

17p or 11q deletion → more advanced disease, respond poorly to conventional tx

88
Q

Most common leukemia in Western hemisphere

A

CLL

89
Q

Si/Sx of CLL

A

Insidious onset - LAN, fatigue, loss of appetite, recurring infections

B symptoms - fever, weight loss, night sweats

Wells syndrome - cutaneous eosinophilia, exaggerated mosquito bite

May have splenomegaly/hepatomegaly

Associated with autoimmune hemolytic anemia or immune thrombocytopenia

90
Q

Diagnostics associated with CLL

A

BONE MARROW ASPIRATE & BIOPSY

Lymphocyte count 5x10^9 w/ <55% atypical cells

LDH rarely elevated

91
Q

Treatment for CLL

A

Early stage = no tx, follow pt

Symptomatic or rapidly progressive = chemo/immunochemo, IBRUTINIB (inhibits Bruton’s tyrosine kinase, crucial for B cell survival)

Consider SCT, palliative care

92
Q

What are the 2 types of lymphoma?

A 
Non-Hodgkins lymphoma (85%)
Hodgkins lymphoma (15%)
93
Q

Which of the 2 lymphomas has a higher survival rate?

A

Hodgkins lymphoma

94
Q

What are the risk factors for NHL?

A
  • Family hx
  • Previous chemo/radiation
  • Immunosuppressive agents and/or organ transplant
  • Exposures to pesticides, hair dyes, dioxins
  • HTLV-1, HIV, EBV, Hep C/B
  • Celiac disease
  • Crohn’s disease
  • Autoimmune disease
95
Q

What are the various B-cell lymphomas?

A 
Diffuse Large B cell lymphoma (DLBCL)
Follicular lymphoma
CLL/Small lymphocytic lymphoma
Burkitt lymphoma
Waldenstrom macroglobulinemia 
Primary CNS lymphoma
96
Q

Which of the B-cell lymphomas are most common?

A

Diffuse large B cell lymphoma

97
Q

Epidemiology of Burkitt lymphoma

A

Associated w/ African children w/ EBV virus

98
Q

Epidemiology of primary CNS lymphoma

A

Associated with immunocompromised (HIV/AIDS)

99
Q

Which NHL are indolent?

How long can they survive without tx?

A

Follicular
CLL/small lymphocytic lymphoma

Survival with untreated dz = years

100
Q

Which NHL are aggressive?

How long can they survive without tx?

A

Diffuse large B cell lymphoma
CNS lymphoma

Survival with untreated dz = months

101
Q

Which NHL are highly aggressive?

How long can they survive without tx?

A

Burkitt’s lymphoma
AIDS-associated lymphoma

Survival with untreated dz = weeks

102
Q

What are si/sx common amongst all the B-cell NHL?

A
  • Painless lymphadenopathy
  • Fever of unknown origin
  • B symptoms (fever, drenching night sweats, weight loss)
  • Hepatosplenomegaly
  • Waldeyer’s ring (extranodal)
  • Mediastinal adenopathy
103
Q

Which of the NHL has waxing/waning painless lymphadenopathy?

A

Follicular

104
Q

Which of the NHL would you see elevated LDH?

A

Aggressive (DLBCL, CNS lymphoma)

Highly aggressive (Burkitt’s)

105
Q

What labs would you need for NHL?

A
  • Elevated LDH (poor prognosis)
  • Elevated uric acid, phosphorus, calcium (lymphoma cell waste products)
  • HIV, Hep B/C
  • Beta-2 microglobulin protein levels (disease burden & tx response)
106
Q

What diagnostic tests are needed for NHL?

A
  • Excisional biopsy of entire, intact node & tissue biopsy
  • BM aspiration
  • CSF if suspect CNS lymphoma
  • Thoracentesis/paracentesis for extranodal involvement
  • PET/CT for staging & tx response
  • MRI to assess CNS involvement
  • Endoscopy if Waldeyer’s ring (extranodal)
107
Q

How do assess the prognosis of NHL?

A

Ann-Arbor staging

108
Q

Ann-arbor staging system

A

Stage I - single lymph node region
Stage II - >1 lymph node region on same side of diaphragm
Stage III - involvement on both sides of diaphragm
Stage IV - Extranodal involvement (e.g. BM)

109
Q

What is the International Prognostic Index (IPI) used for?

A

Determine risk factors for aggressive NHL (i.e. not follicular)

110
Q

What are the risk factors according to IPI?

A 
>60 y/o
Performance status 2-4
Elevated LDH
>1 extranodal site
Ann-Arbor staging III-IV

0-1 → Low
2 → Low-intermediate
3 → High-intermediate
4-5 → High

111
Q

What is FLIPI?

A

Prognosis for follicular NHL

112
Q

What factors are included in FLIPI?

A

NoLASH

Nodes → >4
LDH → elevated
Age → 60+
Ann-Arbor staging → III-IV
Hgb → <12

0-1 Low
2 Intermediate
3+ High

113
Q

Tx for follicular lymphoma

A

Stage I-II → radiation only

Stage III-IV → chemo (CHOP)

114
Q

Tx for DLBCL

A

Stage I-II (<10cm) → CHOP + radiation of involved field

Stage III-IV → CHOP + Rituxan

115
Q

Which of the lymphomas likes to jump around nodes/lymph tissues?

A

NHL

116
Q

What is the difference in age distribution in NHL and HL?

A

NHL typically older pts

HL has bimodal age distribution (25-30 & >55)

117
Q

Risk factors for HL

A
  • Family hx
  • Immunosuppression
  • EBV/mono
  • Smoking

Decreased risk → childhood infections (e.g. varicella, measles, etc.) & breastfeeding

118
Q

What are the 2 major subgroups of HL?

A

Classical Hodgkin’s lymphoma (95%)

Nodular lymphocytic predominant HL (5%)

119
Q

Which lymphoma has Reed-Sternberg cells?

A

Classical Hodgkin’s lymphoma

120
Q

What are Reed-Sternberg cells?

A

Large abnormal lymphocytes that may have >1 nucleus, mostly of B-cell origin - Express CD30 & CD15 surface markers

Found in Classical Hodgkin’s lymphoma

121
Q

What is nodular sclerosing HL

A

Subtype of Classical Hodgkin’s lymphoma → Most common NHL

122
Q

What kind of cells are seen in nodular lymphocytic predominant HL?

A

Lymphocytic & histiocytic cells (L & H cells) → “popcorn cells”

+CD20 but not CD15/30 (R-S cells)

Must use immunohistochemical studies to identify them b/c look similar to NHL, other HL

123
Q

Clinical presentation of HL

A
  • Painless LAN that becomes painful w/ alcohol consumption
  • B symptoms (fever, night sweats, weight loss)
  • Intermittent fever
  • Chest pain, cough, SOB if large mediastinal mass or lung involvement
  • Pruritus
  • Cerebellar degeneration
  • SVC syndrome (HL most commonly above diaphragm) → facial swelling
124
Q

Diagnostic labs of HL

A
  • Elevated LDH (marker of disease burden)
  • Elevated uric acid, phosphorus, calcium (waste products)
  • HIV, Hep B/C serology
  • Pancytopenia
  • ESR → staging of HL
125
Q

Relevant non-lab tests of HL

A

Excisional biopsy of entire lymph node PLUS histology

BM biopsy if elderly, advanced, systemic sx’s, or high-risk histology

PE/CT

126
Q

When would you use modified Ann-Arbor staging?

A

Staging of HL

127
Q

Modified Ann-Arbor Staging, go!

A

Stage I - single lymph node group
Stage II - multiple lymph nodes on SAME SIDE of diaphragm
Stage III - multiple lymph nodes on BOTH sides of diaphragm
Stage IV - extranodal

But WAIT, there’s more!
X → lymph node >10cm
E → extranodal (e.g. BM, pleural effusion w/ +lymphoma cells)
A → absence of B sx’s
B → presence of B sx’s (fever, night sweats, weight loss)

128
Q

3 stages of HL used for treatment

A

Early stage favorable (Stage I-II) → <2 sites, no extranodal, no mediastinal mass >1/3 thoracic diameter, ESR <50 or ESR <30 w/ B sx’s

Early stage unfavorable → Stage I-II that don’t apply to favorable stage

Advanced stage → Stage III-IV or Stage II w/ bulky disease (>10cm)

129
Q

Treatment for classic HL

A

All stages get chemo (ABVD) PLUS radiation

130
Q

Plasma cell disorders

A

Multiple myeloma

Waldenstrom’s macroglobinemia

131
Q

What’s wrong in multiple myeloma?

A

Malignant proliferation of plasma cells (>10% BM) that secrete mostly IgG that is nonfunctional

132
Q

Clinical presentation of multiple myeloma

A
  • Early stages = asymptomatic
  • Fever, malaise
  • Spinal cord compression
  • Hyperviscous blood

CRAB

  • Calcium lvls increased → nausea, fatigue, thirst, constipation, confusion/somnolence, bone lysis
  • Renal failure (light chains deposited in renal tubules) → hypercalcemia, hyperuricemia, recurrent infection
  • Anemia/bleeding (normocytic/chromic)
  • Boney lytic lesions → bone pain ppt by mvmt, pathologic fractures
133
Q

Diagnostics of multiple myeloma

A
  • CBC → pancytopenia, low retic.
  • Rouleaux formation
  • Elevated creatinine, BUN, calcium, uric acid
  • Low albumin
  • Elevated ESR/CRP
  • Elevated LDH
  • 24-hr urine → Bence Jones protein w/ UPEP
  • M spike on SPEP
  • Elevated B2M (marker for tumor burden)
134
Q

Classic triad of multiple myeloma diagnosis

A
  • Lytic bone lesions (x-ray not bone scan)
  • BM plasmacytosis of >10%
  • SPEP w/ M spike or UPEP w/ Bence Jones protein
135
Q

Diagnosis of multiple myeloma

A

Major criteria:

  • Plasmacytosis on tissue bx
  • BM >30% plasma cells
  • SPEP w/ M spike or UPEP w/ Bence Jones protein

Minor criteria:

  • BM 10-30% plasma cells
  • M spike but less than major criteria
  • Lytic bone lesions
  • Abnormally low functional Ig levels

1 major + 1 minor OR 3 minor incl. BM & M spike

136
Q

Staging of multiple myeloma

A

Predicts survival, not prognosis

Stage I → B2M <3.5 → 62 months
Stage II → B2M 3.5-5.5 → 44 months
Stage III → B2M >5.5 → 29 months

137
Q

Tx of multiple myeloma

A
  • Supportive care
  • Chemo +/- autologous SCT (allogenic if relapse)
  • Radiation
  • Maintenance therapy
  • Surgical therapy → kyphoplasty, spinal fusion
138
Q

What is wrong in Waldenstrom’s macroglobulinemia?

A

Excessive plasma cells secreting nonfunctional IgM (much larger than IgG)

139
Q

Clinical presentation of Waldenstrom’s macrogolubinemia?

A
  • Hyperviscous blood
  • B symptoms (fever, weight loss, night sweats)
  • Peripheral neuropathy, fatigue, LAN, recurrent infections
140
Q

Diagnosis of Waldenstrom’s macroglobulinemia

A

High protein via SPEP (UPEP, skeletal survey will be negative)

BM >10% lymphoplasmatic cells

141
Q

Treatment of Waldenstrom’s macroglobulinemia

A

Similar to B-cell lymphoma (rituximab, chemo, ibrutinib)

Clinical Medicine II (1 decks)

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