RF=anti-IgG

 

cryoprecipitation: precipitation of blood proteins at temperatures lower than 37ºC

cryocriet: veergeslagen volume : volume van oorspronkelijk volume plasma/serum

cryofibrinogen: by proteins from plasma

cryoglobulinemie: by proteins from serum (and plasma)

-- cryoprecipitation of proteins from serum and plasma, Ig or Ig/complement, of:

-- SIRS t.g.v. small-to-medium vessel vasculitis due to CG (ook wel “cryoglobulinemic syndrome” and “cryoglobulinemic vasculitis” )

“Meltzer's triad” of palpable purpura, arthralgia, and myalgia is generally seen with polyclonal CGs seen in essential-, viral-, or connective tissue disease-associated CG.

idiopathisch/essential: zonder oorzaak

detectable levels of CGs in a significant proportion of patients with chronic infections and/or inflammation (tientallen procenten), +/- 40% of normal individuals, typically less than 80 mcg/dL, generally insufficient to generate a detectable cryocrit

poor correlation between the cryocrit and clinical symptoms and features

in high CG titer states cryoprecipitates often produce cloud-like structures on peripheral blood smears and may be mistaken for leukocytes or platelets by automated cell differential analyzers

over 0.5 to 1 percent or over 20 to 50 mcg/mL is considered clinically significant

negative test: new specimen after consulting with the laboratory staff

Syndromic diagnosis — elevated cryocrit >1% three to six months + indicators of cryoglobulinemic vasculitis or thrombosis (lower extremity purpura), liefst leukocytoclastic vasculitis or diminished serum C4

LAB/ acute phase reactants, mild normochromic normocytic anemia

the majority of people with CG are asymptomatic

1 in 100,000

PROGNOSIS — The presence of CG per se does not seem to confer a significant morbidity or mortality risk over and above the underlying conditions.

Lymphoproliferative disorders — Secondary lymphoproliferative, generally in type II  5 to 10 years after diagnosis, in less than 5 to 10% (agressief en niet-agressief)

 

 

●Type I CG predominantly affects the skin, kidney, and bone marrow, evgt. polyneuropathy. By contrast, type I CGs more often induce noninflammatory thrombotic lesions, sometimes with evidence of cutaneous infarction or hemorrhage

Brouet (verbinding met symptomen en onderliggende oorzaken klopt niet 100%)

●Type I – isolated monoclonal Ig (typically IgG or IgM, less commonly IgA or free light chains)

few serological complement abnormalities

many type I CGs are present in concentrations greater than 5 or 10 mg/dL (0.05 or 0.1 g/L) with cryocrits may approach 50 percent or may encompass the entire serum volume in type I

5 to 25% of cases

vaak MM/WM

typically causes hyperviscosity by high levels of monoclonal CG

may physically obstruct vessels and/or mediate inflammatory vasculitis

Raynaud phenomenon, digital ischemia, livedo reticularis, and purpura.

None of the patients exhibited central nervous system, pulmonary, cardiac, or gastrointestinal manifestations.

●Mixed cryoglobulinemias:

mixed CGs generally higher concentrations of 1 to 5 mg/dL (0.01 to 0.05 g/L)

reduced levels of total hemolytic complement (CH50) and early complement proteins C1q, C2, and C4, particularly in type II CG as well as in type III CG associated with collagen-vascular disease

often ANA/ENA/ANCA

often constitutional and nonspecific symptoms, such as arthralgias, fatigue, and myalgias

palpable purpura due to cutaneous vasculitis and sensory changes or weakness due to peripheral neuropathy.

pulmonary manifestations may occur

immune complex renal disease

●Type II: polyclonal Ig in association with monoclonal Ig (typically IgM or IgA)

generally between 2 and 7 percent in type II

with RF activity

40 to 60%

often HCV en HIV (d.d. EBV, HBV)

●Type III: polyclonal Ig without a monoclonal Ig component

40-50%

between 1 and 3 percent in type III

some studies suggest that type III levels typically remain less than 1 mg/dL (0.01 g/L)

often secondary to connective tissue diseases

●uitzondering oligoclonal IgM components with or without trace polyclonal Ig responses, often referred to as type II-III [16,17], or biclonal CGs [18,19]

 

 

 

tromboseziekten: TTP, antifosfolipiden, myxoom