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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
The term cryoglobulinaemia refers to the presence in the serum of one (monoclonal cryoimmunoglobulinaemia) or more (mixed cryoglobulinaemia) immunoglobulins, which precipitate at temperatures below 37°C and redissolve on rewarming. Circulating mixed cryoglobulins are often detected in many infectious and systemic disorders. It is characterized by leucocytoclastic vasculitis of small- and medium-sized vessels, and frequent multiple organ involvement due to cryoglobulin-containing immune complexes. Common signs and symptoms are a rash on the lower limbs, arthritis and nerve damage. Cryoglobulinaemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative disease, collagen disease and hepatitis C virus (HCV) infection. Cryoglobulinaemia generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, neuropathy and glomerulonephritis. The diagnosis of cryoglobulinaemia is based on the laboratory demonstration of serum cryoglobulins.
Infectious disease
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
RNA virus (flavivirus) with blood-borne or sexual transmission (former more common). Acute infection is generally mild but leads to chronic carriage in 60–80% of patients. Complications: cryoglobulinaemia. Treatment of chronic hepatitis C infection includes alpha interferon and ribavirin. There is currently no vaccine.
“Primary” Anti-Phospholipid Syndrome
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
The largest series documented was from Asherson et al. comprising 70 patients and the frequency of clinical and serological abnormalities are shown in the accompanying tables (Tables 1 and 2); 54% suffered from episodes of deep venous thrombosis (DVT). These were accompanied by pulmonary thromboembolism in one third. Two patients suffered from pulmonary hypertension (PHT); one had developed this complication following repeated pulmonary thromboembolism; in two others it resembled the “primary” nonthromboembolic variety. IVC thrombosis and a Budd Chiari syndrome were present in two patients. Arterial occlusions were present in 44%, strokes and TIAs being most frequently encountered (50%). Multi-infarct dementia had followed in four. Myocardial infarctions were seen in five patients; occlusion of the aorta in one, multiple visceral arterial occlusions in one, renal infarction in one and renal artery stenosis in one. Subclavian occlusions were present in two and gangrene of the toes occurred in two. Recurrent fetal loss was present in 34%; 46% were ANA positive and antibodies to mitochondria (type MS) were present in 11 of 40 tested. Thirty-two patients had been thrombocytopenic at some stage of their illness. Menorrhagia was encountered in one patient only in whom the platelet count had fallen to 5.10 x 109/1. A false-positive VDRL was present in 17 of 51 patients tested. Coombs positivity occurred in 10, but hemolytic anemia had developed in only three. Six patients demonstrated cryoglobulinemia.
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
Cryoglobulins are serum proteins or protein complexes that precipitate at low temperatures. In type I cryoglobulinaemia, monoclonal IgM precipitates with 10% to 20% of patients with WM having positive test, but less than 5% being symptomatic because of these cryoglobulins [48]. Symptoms of cryoglobulinemia may include acrocyanosis, Raynaud’s phenomenon, palpable purpura, or glomerulonephritis and in view of these complications one should consider PLEX (through a heated circuit) followed by systemic treatment to achieve long-term control [49]. IgM flare may complicate therapy with anti-CD20 MoAbs and can aggravate symptoms, so that preemptive PLEX may be considered [50]. Ibrutinib may be a reasonable therapeutic option, PIs may also reduce IgM levels before initiation of anti-CD20 [51] and BR is another active 1st line treatment option [46,47].
Treatment of systemic necrotizing vasculitides: recent advances and important clinical considerations
Published in Expert Review of Clinical Immunology, 2019
Christian Pagnoux, Arielle Mendel
The diagnosis of CV is usually made by identifying the presence of serum cryoglobulins in a patient with suggestive small-sized–vessel vasculitic manifestations, mostly affecting the peripheral nerves (sensorimotor polyneuropathy or mononeuritis multiplex), skin (purpura, livedo, gangrene) and kidneys (membranoproliferative glomerulonephritis). Type I cryoglobulinemia is usually due to multiple myeloma, Waldenstrom’s macroglobulinemia, B-cell lymphoma, and/or chronic lymphocytic leukemia. HCV-associated CV is one of the most common causes of type II (90% of type II) or type III (70% of type III) cryoglobulinemia [91–93]. After HCV, the most common causes of mixed (type II or III) cryoglobulinemia are autoimmune diseases (mainly Sjögren’s syndrome), lymphoproliferative disorders, and other infections.
Cutaneous small vessel vasculitis
Published in Postgraduate Medicine, 2023
Cryoglobulinemia is the presence of immunoglobulins that precipitate in the cold and resolubilize with warmer temperatures. There are three types based on immunochemical analysis. Monoclonal or type I, mixed or type II, and polyclonal or type III. Vasculitis is only seen in types II and III. They most commonly present with purpura. Half of the patients have peripheral neuropathy and one third have renal involvement. Mixed is generally secondary to hepatitis C infection, and it is considered the main cause accounting for 80–85% of the cases in multiple studies [20]. Type II also can be associated with autoimmune connective tissue disorders and type III with lymphoproliferative disorders [15].