Cryoglobulin jeopardy saganic 3.5

1. Noon conferenceNoon conference
Laura SaganicLaura Saganic
3/5/183/5/18
CryoglobulinemiaCryoglobulinemia
JEOPARDY!JEOPARDY!

2. Jeopardy!Jeopardy!
$100
Cryoglobs It’s complex
Can I get a
witness?
#nerdalert Practica
$200
$300
$400
$500 $500
$400
$300
$200
$100
$500
$400
$300
$200
$100
$500
$400
$300
$200
$100
$500
$400
$300
$200
$100

3. 1 - $1001 - $100
 Cryoglobulin antibodies have this special in vitroCryoglobulin antibodies have this special in vitro
characteristiccharacteristic
 Precipitation <37CPrecipitation <37C

4. 1 - $2001 - $200
 Cryoglobulin preciptation property has little to doCryoglobulin preciptation property has little to do
with this pathophysiologic disease processwith this pathophysiologic disease process
 Immune complex formation occurs in vivo and thisImmune complex formation occurs in vivo and this
actually may not relate to the cryoglobulinactually may not relate to the cryoglobulin
precipitation property. However, severe coldprecipitation property. However, severe cold
exposure, may relate to cold preciptation andexposure, may relate to cold preciptation and
vascular ischemiavascular ischemia

5. 1 - $3001 - $300
 This is the common underlying cause of Type IThis is the common underlying cause of Type I
cryoglobulin, hint, it’s the only monoclonalcryoglobulin, hint, it’s the only monoclonal
composition.composition.
 Hematologic malignancy (WaldenstromHematologic malignancy (Waldenstrom
macroglobulinemia, MM)macroglobulinemia, MM)

6. 1 - $4001 - $400
 90% of people with Type II cryoglobulinemia90% of people with Type II cryoglobulinemia
have this, it’s the antigen sourcehave this, it’s the antigen source
 Hep CHep C

7. 1 - $5001 - $500
 Common underlying cause for a polyclonal IgGCommon underlying cause for a polyclonal IgG
polyclonal IgM composition (Type II)polyclonal IgM composition (Type II)
 Type III Autoimmune disease (SLE, RA)Type III Autoimmune disease (SLE, RA)

8. 2 - $1002 - $100
 Formation of immune complexes lead toFormation of immune complexes lead to
activation of thisactivation of this
 Complement activationComplement activation

9. 2 - $2002 - $200
 Complement is activated by this pathwayComplement is activated by this pathway
 Classical pathwayClassical pathway

10. 2 - $3002 - $300
 This lab test measures the volume-percent ofThis lab test measures the volume-percent of
cryoprecipitates.cryoprecipitates.
 cryocritcryocrit

11. 2 - $4002 - $400
 Type I cryoglobulins rarely form immuneType I cryoglobulins rarely form immune
complexes in vivo, but may cause thiscomplexes in vivo, but may cause this
 HyperviscosityHyperviscosity

12. 2 - $5002 - $500
 Absence of rheumatoid factor argues againstAbsence of rheumatoid factor argues against
thesethese
 Type II and Type III both have rheumatoid factorType II and Type III both have rheumatoid factor
presentpresent

13. 3 - $1003 - $100
 Immune complex vasculitides have thisImmune complex vasculitides have this
characteristic rashcharacteristic rash
 Palpable purpuraPalpable purpura

14. 3 - $2003 - $200
 You won’t find a rash with this type ofYou won’t find a rash with this type of
cryoglobulin disease, immune complexes arecryoglobulin disease, immune complexes are
rarely formedrarely formed
 Type I monoclonal IgM, Waldenstrom, MMType I monoclonal IgM, Waldenstrom, MM

15. 3 - $3003 - $300
 Characteristically, you might find these labCharacteristically, you might find these lab
values with cryoglobulinemic vasculitis, evidencevalues with cryoglobulinemic vasculitis, evidence
of complement activationof complement activation
 C4 disproportionately low relative to C3C4 disproportionately low relative to C3

16. 3 - $4003 - $400
 Hey derm folks, this is the mechanism behindHey derm folks, this is the mechanism behind
palpable purpurapalpable purpura
 Dermal capillaritis, erythrocyte extravasationDermal capillaritis, erythrocyte extravasation

17. 3 - $5003 - $500
 Possible physical exam finding related toPossible physical exam finding related to
cryoglobulins themselvescryoglobulins themselves
 Small vascular infarcts of ears and fingertipsSmall vascular infarcts of ears and fingertips
(cold precipitation)(cold precipitation)

18. 4 - $1004 - $100
 Definitive cryoglobulin treatment requiresDefinitive cryoglobulin treatment requires
clearance of thisclearance of this
 The driving antigenThe driving antigen

19. 4 - $2004 - $200
 Type I cryoglobulins rarely form immuneType I cryoglobulins rarely form immune
complexes, but they can do this to the bloodcomplexes, but they can do this to the blood
 hyperviscocityhyperviscocity

20. 4 - $3004 - $300
 ***DAILY DOUBLE******DAILY DOUBLE***
 This molecule is generated through the activation of theThis molecule is generated through the activation of the
classical pathway and tags pathogens and immuneclassical pathway and tags pathogens and immune
complexes for phagocytosis (opsinization)complexes for phagocytosis (opsinization)
 C3bC3b

21. 4 - $4004 - $400
 List 3 treatment options for severe multiorganList 3 treatment options for severe multiorgan
cryoglobuminemiacryoglobuminemia
 Glucocorticoids, cyclophosphamide,Glucocorticoids, cyclophosphamide,
plasmapheresis, and rituxanplasmapheresis, and rituxan

22. 4 - $5004 - $500
 Glomerular nephritis typically occurs with thisGlomerular nephritis typically occurs with this
type of cryoglobuminemiatype of cryoglobuminemia
 Type IIType II

23. 5 - $1005 - $100
 68M 6mo progressive DOE, LEE, burning pain with68M 6mo progressive DOE, LEE, burning pain with
numbness in his extremities. No PMH, no meds, recentnumbness in his extremities. No PMH, no meds, recent
labs normal. PE 140/70. Hepatosplenomegaly. Impairedlabs normal. PE 140/70. Hepatosplenomegaly. Impaired
touch and vibration sense in glove and stockingtouch and vibration sense in glove and stocking
distribution. LE edema.distribution. LE edema.
 Cr 1.6, electrolytes normal, BG 98, albumin 2.8,Cr 1.6, electrolytes normal, BG 98, albumin 2.8,
urinalysis 3+ protein, no blood or cells, urine protein-urinalysis 3+ protein, no blood or cells, urine protein-
creatinine ratio 4800mg/gcreatinine ratio 4800mg/g
 Which of the following is the most likely diagnosis on renal biopsy?Which of the following is the most likely diagnosis on renal biopsy?
A) AL amyloidosisA) AL amyloidosis
B) Diabetic nephropathyB) Diabetic nephropathy
C) Myeloma nephropathyC) Myeloma nephropathy
D) Primary focal segmental glomerulosclerosisD) Primary focal segmental glomerulosclerosis

24. 5 - $2005 - $200
 50M fatigue, joint pain, and skin lesions. 2 weeks ago fever and sore throat. Dark50M fatigue, joint pain, and skin lesions. 2 weeks ago fever and sore throat. Dark
red spots on ankles and shins, intermittent abdominal pain, crampy. PE 170/84,red spots on ankles and shins, intermittent abdominal pain, crampy. PE 170/84,
periumbilical tenderness, joint tendernessperiumbilical tenderness, joint tenderness
 Labs Albumin 3.1, C3/C4 nl, Cr 1.8, HepB HCV neg. Urine 3+ blood, 2+ protein;Labs Albumin 3.1, C3/C4 nl, Cr 1.8, HepB HCV neg. Urine 3+ blood, 2+ protein;
50-100 erythrocytes, few erythrocyte casts, urine protein-cr ratio 2200mg/g50-100 erythrocytes, few erythrocyte casts, urine protein-cr ratio 2200mg/g
 Most likely diagnosis?Most likely diagnosis?
A)A) Cryoglobulinemic vasculitisCryoglobulinemic vasculitis
B)B) EndocarditisEndocarditis
C)C) IgA vasculitisIgA vasculitis
D)D) SLESLE

25. 5 - $3005 - $300
 46F 1 wk polyarthritis in hands, wrists and knees with a46F 1 wk polyarthritis in hands, wrists and knees with a
rash. PE symmetric tenderness, warmth, erythema andrash. PE symmetric tenderness, warmth, erythema and
swelling of wrists, PIP and MCP and knees bilaterallyswelling of wrists, PIP and MCP and knees bilaterally
 Labs ERS 63, ALT 1050, AST 800, Cr 1.0Labs ERS 63, ALT 1050, AST 800, Cr 1.0
 Which of the following is the most likely diagnosis?Which of the following is the most likely diagnosis?
A)A) Autoimmune hepatitisAutoimmune hepatitis
B)B) HemochromatosisHemochromatosis
C)C) HBV-associated arthritisHBV-associated arthritis
D)D) CryoglobuminemiaCryoglobuminemia

26. 5 - $4005 - $400
 32F new rash on legs. 4d ago pyelo treated with32F new rash on legs. 4d ago pyelo treated with
TMP/SMX. PE vitals normal, MSK exam no evidence ofTMP/SMX. PE vitals normal, MSK exam no evidence of
swelling. Palpable purpura on LE. Which of theswelling. Palpable purpura on LE. Which of the
following is the most appropriate next step?following is the most appropriate next step?
A)A) Discontinue TMP/SMXDiscontinue TMP/SMX
B)B) Initiate prednisoneInitiate prednisone
C)C) Measure antihistone antibodiesMeasure antihistone antibodies
D)D) Obtain skin biopsyObtain skin biopsy

27. 5 - $5005 - $500
 39M LE rash 3 wks. PE vitals normal. Palpable purpura in bilateral extremities.39M LE rash 3 wks. PE vitals normal. Palpable purpura in bilateral extremities.
 Labs ESR 66, C3 nl, C4 low, Cr 2.1, Rf pos, HCV positive, Serum proteinLabs ESR 66, C3 nl, C4 low, Cr 2.1, Rf pos, HCV positive, Serum protein
monoclonal spike in IgG.monoclonal spike in IgG.
Which of the following is most likely to establish the diagnosis?Which of the following is most likely to establish the diagnosis?
A)A) anti-CCP antibodyanti-CCP antibody
B)B) Anti-glomerular basement membrane antibody levelsAnti-glomerular basement membrane antibody levels
C)C) ANAANA
D)D) P-ANCAP-ANCA
E)E) Serum cryoglobulinSerum cryoglobulin