Hematology/Oncology Flashcards
1
Q
What is wrong in hemophilia?
A
Deficiency of factor VIII or IX (intrinsic pathway) -> insufficient thrombin production
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
3
Q
Which hemophilias are recessive X-linked disorders?
A
Hemophilia A & B
Hemophilia C is autosomal disorder
4
Q
What are the symptoms of hemophilia C?
A
Mild spontaneous bleeding only - just need ppx for surgical procedures
5
Q
What are the different severities of hemophilia?
A
Severe: <1% active factor
Moderate: 1-5% active factor
Mild: 5-40% active factor
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
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)
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
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
10
Q
What do you if a hemophilia pt experiences an acute bleeding episode?
A
Raise the factor level to >50
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)
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)
13
Q
What is the daily requirement of vitamin K?
A
100-200mcg/day
14
Q
How does the body get vitamin K?
A
Mainly from diet - absorbed in terminal ileum
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)
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)
17
Q
How do you treat vitamin K deficiency?
A
Vitamin K supplementation (diet or PO/IV)
18
Q
What is DIC?
A
Consumption of platelets d/t microvascular thrombi formation
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
20
Q
What is the pathophysiology behind DIC?
A
Excessive plasmin (degrades fibrin clots) or excessive thrombin formation
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
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)
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
24
Q
What is polycythemia vera?
A
Chronic acquired myeloproliferative neoplasm causing overproduction of abnormal BM products (esp. RBC) → mutation in JAK2
25
What are the consequences of polycythemia vera?
Hyperviscosity of blood → Increased cardiac workload
Ischemia/thrombosis of organs
26
What are the types of polycythemia vera?
Primary → mutated JAK2 that makes RBC without EPO activation
Secondary → EPO-driven production of RBCs (no JAK2 mutation)
27
What can cause secondary polycythemia vera?
- Hypoxia (high altitude, smoking, sleep apnea)
- EPO-secreting tumors (RCC, pheo)
- Adrenal (adenoma, Cushing's)
- Renal (polycystic kidney disease, hydronephrosis)
- Testosterone
28
How would a pt with polycythemia vera present?
- 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
29
Labs related to polycythemia vera
- Elevated Hgb, Hct, WBC, platelets
- Low serum EPO
- BM histopathology shows JAK2 mutation
30
Treatment for polycythemia vera
- Phlebotomy plus low-dose ASA
- If high risk, non-compliant on phlebotomy, or progressive (splenomegaly, leukocytosis, thrombocytosis) → hydroxyurea +/- interferon
31
How are platelets formed?
Thrombopoiesis in BM from megakaryocytes → shed directly into BM
Each megakaryocyte produces 50,000-10,000 platelets
32
How long do platelets live? Where do they go to die?
5-9 days → Old platelets are destroyed by spleen via phagocytosis & liver via Kupffer cells
33
What is thrombocytopenia?
<150,000/mcL
Note: 2.5% normal pop. has plt count <150,000
34
What is the normal range for platelet levels?
150,000-450,000/mcL
35
What are the 4 general causes of thrombocytopenia?
- Decreased plt production
- Increased plt destruction
- Dilutional thrombocytopenia → big transfusion of pRBC
- Distributional thrombocytopenia → cirrhosis, portal htn, splenomegaly
36
What can cause decreased platelet production, leading to thrombocytopenia?
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
37
What can cause increased plt destruction, leading to thrombocytopenia?
- 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
38
Clinical presentation of thrombocytopenia
Can be asymptomatic until plt count <100,000/mcL
Bleeding, ecchymosis, purpura, petechiae
Spontaneous bleeding if <20,000/mcL
39
What's wrong in von Willebrand disease?
Qualitative/quantitative deficiency of vWF, which is required for platelet adhesion → bleeding
40
What are the types of vWD?
- 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
41
What are some causes of acquired vWD?
- Cardiac valvulopathies → mechanical shearing of vWF
- DIC → degradation by plasmin
- Hypothyroidism → reduced synthesis
- Autoimmune diseases
42
What diagnostics are involved with vWD?
- vWF antigen assay → evaluate quantity/quality
- Factor VIII levels
- Normal PT but variable prolongation of aPTT (insufficient VIII)
43
Treatment for vWD
- Desmopressin → increases vWF/VIII release from endothelial cells; used for ppx and tx
- Transfusion of vWF-containing VIII conc./FFP
44
What's wrong in idiopathic thrombocytopenic purpura (ITP)?
Thrombocytopenia with normal BM functioning (diagnosis of exclusion)
45
What is the pathophysiology behind idiopathic thrombocytopenic purpura (ITP)?
Autoimmune - antibodies against plt antigens & IgG autoantibodies that damage megakaryocytes
Inadequate production d/t deficiency of TPO
46
2 types of ITP
Acute → <2 months, s/p infection in children
Chronic ITP → >6 months
47
At what plt count would a pt be at risk for life-threatening bleed?
<20,000/mcL
48
Treatment for ITP
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)
49
What's wrong in thrombotic thrombocytopenic purpura (TTP)?
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
50
Clinical presentation of TTP
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
```
51
Diagnosis criteria for TTP
3 or more FATRN sx's with schisctocytes
Normal PTT
52
Tx of TTP
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
53
Cells that arise from lymphoid progenitor
NK cells
T cells
B cells
54
Cells that arise from myeloid progenitor
RBC
WBC
platelets
55
Normal WBC range
4,000-11,000
56
Leukopenia
<4,000 WBC
57
Leukocytosis
>11,000 WBC
58
Normal WBC differential (% of each cell type)
```
Neutrophils 50-70%
Lymphocytes 20-40%
Monocytes 5-10%
Eosinophils 1-3%
Basophils 0-1%
```
59
Categorization of hematologic malignancies
Acute vs. chronic
| Lymphoid vs. myeloid
60
What are lymphomas anyway?
Discrete, well-defined mass in bone marrow/lymphoid tissue formed by hematopoietic precursor cells (blasts)
61
Classic symptoms of any lymphoma
PAINLESS LYMPHADENOPATHY
Fever of unknown origin
Weight loss
62
Acute vs chronic leukemias
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
Epidemiology of AML
More common in adults
64
Etiology of AML
- 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
Clinical presentation of AML
Non-specific but ABRUPT-ONSET (fatigue, anemia, anorexia, weight loss, bleeding, etc.)
Splenomegaly, LAN, sternal tenderness, gum hypertrophy, papilledema
66
Work-up findings for AML
BONE MARROW ASPIRATE & BIOPSY → >20% blasts
Auer rods (M2, M3, M4)
Leukocytosis, blastemia, thrombocytopenia, anemia
67
Treatment for AML
Immediate inpatient chemo
| SCT if relapse or recurrence
68
3 Phases of CML
Chronic (<10% blasts) → asymptomatic, most are diagnosed at this stage
Accelerated (≥15% blasts)
Blast → ≥30% blasts + extramedullary disease → poor prognosis
69
Clinical presentation of CML
Lymphadenopathy
Mild hepatomegaly
Splenomegaly (in blast crisis)
Myeloid sarcoma
70
Work-up findings of CML
BONE MARROW ASPIRATE & BIOPSY
Cytogenics → Ph chromosome
FISH → bcr/abl arrangement
CBC w/ diff → incr. immature/mature granulocytes (neutrophils, basophils, eosinophils), variable platelets
71
What's the big deal about Philadelphia chromosome?
Abl gene from chrom. 9 inserts into bcr gene in chrom. 22 → bcr/abl oncogene
Associated with CML
72
Tx of CML
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
Indicators of poor prognosis of CML
- Progression to blast phase
- Older age
- Severe anemia
- Negative Ph chromosome
- Thrombocytopenia
- Hepatomegaly
- Splenomegaly
74
Epidemiology of ALL
Bimodal distribution
| Most common childhood cancer
75
Risk factors for ALL
- Previous cancer tx
- Exposure to radiation
- Genetic disorders (Down syndrome, Klinefelter)
- Sibling w/ ALL
- Varicella in childhod ALL
76
Clinical presentation for ALL
Bone marrow failure → malaise, fatigue, bleeding, bruising, secondary infections
B symptoms → fever, weight loss, night sweats
CNS INVOLVEMENT → cranial neuropathies, meningeal infiltration
77
Work-up for ALL
BONE MARROW ASPIRATE & BIOPSY
LP to assess CNS involvement
CBC w/ diff → lymphoblasts, normal/decr. WBCs, anemia, thrombocytopenia
78
Main tx for ALL
Chemo w/ CNS prophylaxis
79
What are the 4 components of chemo for ALL tx?
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
What happens in induction phase for ALL tx?
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
What happens in consolidation phase for ALL tx?
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
What happens in maintenance phase of ALL tx?
Maintain remission
Lower doses of chemo for 18-24 months
T-cell and mature B-cell ALL may not need maintenance
83
What happens in CNS ppx phase of ALL tx?
Begins during induction phase and continues through entire tx regimen
Intrathecal chemo via LP or Onmaya reservoir (intraventricular)
84
What additional tx options can be added onto chemo for ALL tx?
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
Indicators of good prognosis for ALL
```
<50,000 WBC
Age 1-10
Female
B-cell blasts
Huge reduction of blasts at day 7 of induction
```
86
Indicators of poor prognosis for ALL
```
>50,000 WBC
<1 or >10 y/o
Male
T-cell blasts
Mild reduction of blasts at day 7 of induction
```
87
Markers involved with CLL
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
Most common leukemia in Western hemisphere
CLL
89
Si/Sx of CLL
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
Diagnostics associated with CLL
BONE MARROW ASPIRATE & BIOPSY
Lymphocyte count 5x10^9 w/ <55% atypical cells
LDH rarely elevated
91
Treatment for CLL
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
What are the 2 types of lymphoma?
```
Non-Hodgkins lymphoma (85%)
Hodgkins lymphoma (15%)
```
93
Which of the 2 lymphomas has a higher survival rate?
Hodgkins lymphoma
94
What are the risk factors for NHL?
- 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
What are the various B-cell lymphomas?
```
Diffuse Large B cell lymphoma (DLBCL)
Follicular lymphoma
CLL/Small lymphocytic lymphoma
Burkitt lymphoma
Waldenstrom macroglobulinemia
Primary CNS lymphoma
```
96
Which of the B-cell lymphomas are most common?
Diffuse large B cell lymphoma
97
Epidemiology of Burkitt lymphoma
Associated w/ African children w/ EBV virus
98
Epidemiology of primary CNS lymphoma
Associated with immunocompromised (HIV/AIDS)
99
Which NHL are indolent?
How long can they survive without tx?
Follicular
CLL/small lymphocytic lymphoma
Survival with untreated dz = years
100
Which NHL are aggressive?
How long can they survive without tx?
Diffuse large B cell lymphoma
CNS lymphoma
Survival with untreated dz = months
101
Which NHL are highly aggressive?
How long can they survive without tx?
Burkitt's lymphoma
AIDS-associated lymphoma
Survival with untreated dz = weeks
102
What are si/sx common amongst all the B-cell NHL?
- Painless lymphadenopathy
- Fever of unknown origin
- B symptoms (fever, drenching night sweats, weight loss)
- Hepatosplenomegaly
- Waldeyer's ring (extranodal)
- Mediastinal adenopathy
103
Which of the NHL has waxing/waning painless lymphadenopathy?
Follicular
104
Which of the NHL would you see elevated LDH?
Aggressive (DLBCL, CNS lymphoma)
Highly aggressive (Burkitt's)
105
What labs would you need for NHL?
- 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
What diagnostic tests are needed for NHL?
- 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
How do assess the prognosis of NHL?
Ann-Arbor staging
108
Ann-arbor staging system
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
What is the International Prognostic Index (IPI) used for?
Determine risk factors for aggressive NHL (i.e. not follicular)
110
What are the risk factors according to IPI?
```
>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
What is FLIPI?
Prognosis for follicular NHL
112
What factors are included in FLIPI?
NoLASH
```
Nodes → >4
LDH → elevated
Age → 60+
Ann-Arbor staging → III-IV
Hgb → <12
```
0-1 Low
2 Intermediate
3+ High
113
Tx for follicular lymphoma
Stage I-II → radiation only
| Stage III-IV → chemo (CHOP)
114
Tx for DLBCL
Stage I-II (<10cm) → CHOP + radiation of involved field
Stage III-IV → CHOP + Rituxan
115
Which of the lymphomas likes to jump around nodes/lymph tissues?
NHL
116
What is the difference in age distribution in NHL and HL?
NHL typically older pts
HL has bimodal age distribution (25-30 & >55)
117
Risk factors for HL
- Family hx
- Immunosuppression
- EBV/mono
- Smoking
Decreased risk → childhood infections (e.g. varicella, measles, etc.) & breastfeeding
118
What are the 2 major subgroups of HL?
Classical Hodgkin's lymphoma (95%)
Nodular lymphocytic predominant HL (5%)
119
Which lymphoma has Reed-Sternberg cells?
Classical Hodgkin's lymphoma
120
What are Reed-Sternberg cells?
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
What is nodular sclerosing HL
Subtype of Classical Hodgkin's lymphoma → Most common NHL
122
What kind of cells are seen in nodular lymphocytic predominant HL?
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
Clinical presentation of HL
- 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
Diagnostic labs of HL
- Elevated LDH (marker of disease burden)
- Elevated uric acid, phosphorus, calcium (waste products)
- HIV, Hep B/C serology
- Pancytopenia
- ESR → staging of HL
125
Relevant non-lab tests of HL
Excisional biopsy of entire lymph node PLUS histology
BM biopsy if elderly, advanced, systemic sx's, or high-risk histology
PE/CT
126
When would you use modified Ann-Arbor staging?
Staging of HL
127
Modified Ann-Arbor Staging, go!
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
3 stages of HL used for treatment
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
Treatment for classic HL
All stages get chemo (ABVD) PLUS radiation
130
Plasma cell disorders
Multiple myeloma
| Waldenstrom's macroglobinemia
131
What's wrong in multiple myeloma?
Malignant proliferation of plasma cells (>10% BM) that secrete mostly IgG that is nonfunctional
132
Clinical presentation of multiple myeloma
- 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
Diagnostics of multiple myeloma
- 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
Classic triad of multiple myeloma diagnosis
- Lytic bone lesions (x-ray not bone scan)
- BM plasmacytosis of >10%
- SPEP w/ M spike or UPEP w/ Bence Jones protein
135
Diagnosis of multiple myeloma
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
Staging of multiple myeloma
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
Tx of multiple myeloma
- Supportive care
- Chemo +/- autologous SCT (allogenic if relapse)
- Radiation
- Maintenance therapy
- Surgical therapy → kyphoplasty, spinal fusion
138
What is wrong in Waldenstrom's macroglobulinemia?
Excessive plasma cells secreting nonfunctional IgM (much larger than IgG)
139
Clinical presentation of Waldenstrom's macrogolubinemia?
- Hyperviscous blood
- B symptoms (fever, weight loss, night sweats)
- Peripheral neuropathy, fatigue, LAN, recurrent infections
140
Diagnosis of Waldenstrom's macroglobulinemia
High protein via SPEP (UPEP, skeletal survey will be negative)
BM >10% lymphoplasmatic cells
141
Treatment of Waldenstrom's macroglobulinemia
Similar to B-cell lymphoma (rituximab, chemo, ibrutinib)
