Hyperviscosity syndrome

Classification according to ICD-10
R70.1	Abnormal plasma viscosity, hyperviscosity syndrome, change in plasma viscosity
ICD-10 online (WHO version 2019)

The hyperviscosity syndrome (abbreviation HVS; English hyperviscosity syndrome ) is a clinical complex of symptoms caused by an increased blood viscosity , which corresponds to a reduction in the flowability of the blood.

pathology

The viscosity of the blood depends, among other things, on the concentration of the paraproteins dissolved in its liquid phase ( plasma ) and their physical and chemical properties.

In the case of IgG , a linear increase in the viscosity of the blood can be observed when the concentration of the paraproteins of the IgG class exceeds 120 g / l.

In the case of IgA and certain subgroups of IgG, insoluble precipitates arise in the plasma through the formation of disulfide bridges .

In the IgM of Waldenstrom is a large molecule composed of five Y-shaped subunits. In this disease, an IgM concentration of 40 g / l is sufficient for the development of HVS.

With IgG _{3 in} particular, reversible complexes can form at low temperatures and high concentrations, which can also increase the viscosity of the blood. One speaks of cryoglobulins , which can lead to symptoms similar to Raynaud's disease in the extremities .

Epidemiology

The hyperviscosity syndrome caused by paraproteins is found in malignant diseases such as multiple myeloma and Waldenström's disease. HVS can also occur in a number of benign diseases, including Felty's syndrome , lupus erythematosus and rheumatoid arthritis .

The HVS occurs in up to 10% dF in multiple myeloma and in up to 30% dF in M. Waldenström.

clinic

In general, patients complain of fatigue, weakness and shortness of breath. An anemia caused by mucosal and nosebleeds because it to disruption of platelet function occurs. This platelet dysfunction is based on an impairment of the receptors necessary for coagulation, since the platelet surface is coated by the paraproteins and there can be an interaction with fibrin formation . The result is a symptom that is comparable to a microangiopathy . In bedridden patients, the risk of thrombosis and thromboembolism can be significantly increased.

manifestation	Symptoms
General	Weakness fatigue loss of appetite
Coagulation	Epistaxis Bleeding of the oral mucosa Prolonged bleeding time after injuries Anemia Symptoms of thrombosis and thromboembolism
Central nervous system	Headache dizziness somnolence to coma epileptic seizures sensory disorders
eye	Loss of vision Diplopia congestion of the retinal veins
ear	Hearing loss
Cardiovascular	Heart failure angina pectoris
kidney	Decrease in kidney function due to glomerular obstruction

laboratory

Typical findings on the HVS are a very greatly increased ESR with more than 100 mm / h, in the serum electrophoresis show paraproteins . The measurement of the blood viscosity with the capillary viscometer indicates an increase in the values. Rare, important for the differential diagnosis, is evident in the electrolyte a pseudohyponatraemia because the Para proteins bind the free water of the plasma.

diagnosis

Difficulties in taking blood due to blocked cannulas can provide an initial indication of the HVS. The HVS usually only occurs with certain underlying diseases. Laboratory tests as well as blood counts and bone marrow tests can be used to confirm the suspected diagnosis .

Therapy and prognosis

The symptomatic (and usual) therapy for hyperviscosity syndrome using paraproteins is plasma exchange using plasmapheresis . A cell separator separates cellular blood components from the blood plasma and replaces it (with pooled foreign donor plasma or human albumin). Plasma exchange is only indicated in emergency situations (coma, epileptic seizures, heart failure), in which case up to 6 liters of plasma can be exchanged. Otherwise, one or two plasmapheresis per week is sufficient to remove the excess immunoglobulin from the blood.

The curative therapy is to treat the underlying disease, which also determines the prognosis.

literature

• H.-W. Baenkler among others: internal medicine. Thieme Verlag, 2001, ISBN 3-13-128751-9 .

Free access to PubMed Central

• JD Capra, HG Kunkel: Aggregation of gamma-G3 proteins: relevance to the hyperviscosity syndrome. In: J Clin Invest. 1970 Mar; 49 (3), pp. 610-621. PMID 5415687
• NA Buskard et al .: Plasma exchange in the long-term management of Waldenström's macroglobulinemia. In: Can Med Assoc J. 1977 Jul 23; 117 (2), pp 135-137. PMID 406031

Individual evidence

1. ↑ see also: Thomas Deller: Blood. ( Memento of the original from March 4, 2016 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. Script. J. W. Goethe University, Frankfurt am Main, PDF file, accessed on May 21, 2012. 
2. ↑ Herold: Internal Medicine. Self-published, Cologne 2002, OCLC 313938733 .

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