22 - 144 - CRYOGLOBULINEMIA/ CRYOFIBRINOGENEMIA Flashcards
Classical underlying disease in type 2 cryoglobulinemia
a. Hep a
b. Hep b
c. Hep c
d. Hematologic malignancy
C
Meltzer triad is composed of, except
a. purpura
b. bleeding
c. arthralgias
d. weakness
B
most frequently encountered malignancy in type 1 cryoglobulinemia
a. Hodgkin lymphoma
b. non-hodgkin lymphoma
c. ALL
d. AMLB
B
the ff. medications are used for immunosuppressive combination therapy, except:
a. steroids
b. rituximab
c. cyclophosphamide
d. chlorambucil
B
TOC for patients with non-severe cases of cryofibrinogenemia
a. Oral stanozolol
b. Streptokinase
c. Prednisone with low dose aspirin
d. Colchicine
C
circulating immunoglobulins found in both serum and plasma that reversibly precipitate or gel upon cold exposure
Cryoglobulins
result from precipitation of fibrinogens on cold exposure and are detectable only in plasma samples, not serum
Cryofibrinogens
hallmark of cryoglobulinemia
purpura at distal sites
Identify the type of cryoglobulins
- consist of a** single monoclonal immunoglobulin **(typically IgG) or light chain
- They occur with hematologic malignancies such as multiple myeloma
- If symptomatic, they present with a noninflammatory occlusive vasculopathy.
Type I cryoglobulins
Identify the type of cryoglobulins
They consist of a monoclonal immunoglobulin (typically IgM) complexed with a polyclonal immunoglobulin (typically IgG)
Type II cryoglobulins
Identify the type of cryoglobulins
- consist of** purely polyclonal** immunoglobulin complexes
Type III cryoglobulins
Identify the type of cryoglobulins
They are also referred to as mixed cryoglobulinemias
Types II and III
mixed cryoglobulinemias are caused by what infection?
chronic hepatitis C virus (HCV) infection in >90% of cases
this type of cryoglobulins may fix complement
Type II and Type III cryoglobulins may fix complement.
Complement components are not routinely found in Type I cryoprecipitates.
classical underlying disease of Type II cryoglobulins
HCV infection
Infarction, hemorrhagic crusts, ulcers, and lesions on the head and oral or nasal mucosa are relatively more common in this type of cryoglobulinemia
Type I
most common presentation of Type II cryoglobulinemia and particularly affects the lower extremities
Intermittent orthostatic palpable purpura, frequently observed late in the afternoon when highest cryoglobulin concentrations
The face and the trunk, with the exception of the lower abdomen, are usually spared.
histopathologic hallmark of mixed cryoglobulinemia
Leukocytoclastic vasculitis
most common extracutaneous manifestations in patients with mixed cryoglobulinemic vasculitis
weakness and arthralgias or arthritis
Arthralgias classically affect the proximal interphalangeal and metacarpophalangeal joints of the hands, the knees, and ankles
“Meltzer triad”
purpura, arthralgias, and weakness
most frequently encountered hematologic malignancy in Type I cryoglobulinemia
Non-Hodgkin B-cell lymphoma
reserved for instances of mixed cryoglobulinemia in the absence of a well-defined underlying disease and accounts for only a minority of cryoglobulinemic patients
essential cryoglobulinemia
temperature needed for serum storage for inspecition of precipitate
4 degrees
how many % cryokit is considered to be positive
A cryocrit ≥2% is considered to be positive
- For calculation of cryocrit (volume of packed cryoglobulins as percentage of original serum volume), the cryoprecipitate has to be spun in a graded (Wintrobe) tube
- Cryocrit levels usually do not correlate with severity and prognosis of disease.
most common cause of death in HCV patients with mixed cryoglobulinemia
Infection
followed by complications related to liver and cardiac involvement
organs most vulnerable to complications
nervous system and the kidneys
poor prognostic sign in patients with cryoglobulinemic vasculitis
Renal involvement
treatment of choice for Type I cryoglobulinemia
Etiologic treatment
current gold standard of therapy for patients with HCV-ASSOCIATED TYPE II AND III CRYOGLOBULINEMIA IN PATIENTS WITH MILD TO MODERATE DISEASE
PEGylated interferon α-2b and ribavirin
most frequent clinical presentation of cryofibrinogenemia
Palpable purpura with underlying leucocytoclastic vasculitis
significant predisposing factor for thrombotic events of cryofibrinogenemia
high cryofibrinogen plasma
