Hyperviscosity

Chronic transfusion is indicated to prevent stroke and stroke recurrence in children. Transfusion frequency is usually every 3 to 4 weeks and should be adjusted to maintain HbS of less than 30% of total hemoglobin. The optimal duration is unknown. Risks include alloimmunization, hyperviscosity, viral transmission (requiring hepatitis A and B vaccination), volume and iron overload, and transfusion reactions. 

Therapy, since neither white cell nor platelet count are raised, is venesection at whatever interval necessary to maintain packed cell volume between 40% and 45%. If this continues long enough iron stores will be depleted and the time between consecutive blood collections is extended. This deficiency state should not be corrected except in the very rare circumstance of paradoxical hyperviscosity where symptoms are related to the poor deformability of the hyprchromic and microcytic red cells in the microcirculation. Where possible, underlying medical illnesses such as cardiopulmonary disease, should be corrected and the patient advised to stop smoking. In those individuals where respiratory function is normal during the day it is necessary to repeat this whilst asleep at which time the hypoxic stimulus may be revealed. 

Siciliano, L., Tarantino, P., Longobardi, E, Rago, V., De Stefano, C., and Carpino, A., Impaired seminal antioxidant capacity in human semen with hyperviscosity or oligoasthenozoospermia. J. Androl. 22, 798-803 (2001). 

Unless M protein concentrations exceed 60 g/L, patients with multiple myeloma do not develop hyperviscosity syndrome. Despite the presence of plasma cells in bone marrow exceeding 10% and the presence of M protein exceeding 25 g/L, nearly 15% of patients with multiple myeloma are asymptomatic. However, asymptomatic patients presenting with IgA myeloma protein and M protein concentrations exceeding 30 g/L and Bence Jones-protein excretion in excess of 50 mg/day in presence of a lytic bone lesion can progress to multiple myeloma earlier than other, asymptomatic patients (Wl). 

The concentration of monoclonal IgM in serum approximates 30 g/L or greater (K37). A significant number of patients excrete monoclonal light chains in urine, with a predominance of k over A. light chains (K37). An increase in monoclonal IgM contributes to an increase in serum viscosity. In one study 15 patients with WM whose M protein concentrations ranged from 43 to 80 g/L (median 55 g/L), had serum viscosity greater than 4.0 cP (K37). Rouleaux formation in hyperviscosity syndrome results in an increase in erythrocyte sedimentation rate (ESR). 

Common risk factors for developing branch retinal vein thrombosis (BRVT) and central retinal vein thrombosis (CRVT) include increased plasma fibrinogen, diabetes, decreased exercise, hypertension, and hyperviscosity (205). Sickle cell anemia, polycythemia vera, and other proliferative disorders may also lead to this syndrome. 

Pll. Pope, R. M., Fletcher, M. A., Mamby, A., and Shapiro, C. M., Rheumatoid arthritis associated with hyperviscosity syndrome and intermediate complex formation. Arch. Intern. Med. 135, 281-285 (1975). 

There is no clear evidence of specific central nervous system adverse effects due to G-CSF. In one patient, neurological symptoms, such as blurred vision, weakness, and headache, were attributed to G-CSF-induced extreme hyperleukocytosis with subsequent hyperviscosity (SEDA-21, 377). Encephalopathy, cortical blindness and seizures have also been mentioned in single case report (SEDA-21, 377). 

The authors of both reports suggested that the hemiplegia had been caused either by transient hyperviscosity or by vasospasm. 

Intensive plasma exchange has been studied in the treatment of numerous diseases, above all in Waldenstrom s macroglobulinemia, hypercholesterolemia, hyperviscosity syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, myasthenia... 

A 46-year-old man developed a Q-wave inferior and a right ventricular myocardial infarct with postinfarction angina after the third cycle of vincristine -I- doxorubicin for multiple myeloma. The patient had no risk factors for ischemic heart disease, except for a positive smoking history, nor for hyperviscosity (24). 

IgE (0.1% myelomatosis). The two recorded patients (01) initially had no bone lesions (but Johansson s case did later), and both seemed to have a tendency to plasma cell leukemia and hyperviscosity, perhaps because many of our few normal IgE precursors may circulate in the blood stream. 

V2. Virella, G., and Hobbs, J. R., Heavy chain typing in IgG monoclonal gammo-pathies with special reference to cases of serum hyperviscosity and cryoglobu-linaemia. Clin. Exp. Immunol. 9, 973-980 (1971). 

The unmeasured anion is commonly known as the anion gap, which is normally 12 4 mEq/L. This value is useful in assessing the acid-base status of a patient and in diagnosing metabolic acidosis. Disorders that cause a high anion gap are metabolic acidosis, dehydration, therapy with sodium salts of strong acids, therapy with certain antibiotics (e.g., carbenicillin), and alkalosis. A decrease in the normal anion gap occurs in various plasma dilution states, hypercalcemia, hypermagnesemia, hypernatremia, hypoalbuminemia, disorders associated with hyperviscosity, some paraproteinemias, and bromide toxicity. 

This uncommon lymphoma morphologically consists of a mixture of lymphoid cells, plasma cells, and cells sharing the characteristics of both lymphocytes and plasma cells. Mast cells are quite prominent. Plasma-cytoid features may take the form of Dutcher bodies, as well as Russell bodies, which may be enhanced by staining with PAS. The typical presentation for this lymphoma is bone marrow involvement with little nodal or spleen involvement. These lymphomas typically present with a monoclonal gammopathy of IgM and its associated hyperviscosity and/or cryoglobulinemia (Waldenstrom macroglobulinemia). An association with hepatitis C has been noted with this lymphoma. 

D-penicillamine is so named because it was first isolated as an amine, from the degradation products of penicillin by Abraham et al [87]. Later studies showed the characteristic chemical behavior of D-penicillamine which involve three types of reactions, formation of disulphide links, formation of thiazolidine rings, and formation of metal complexes and chelates [67]. It was first used in 1956 in the treatment of Wilson s disease [88]. D-penicillamine has since been used in the treatment of many diseases, such as cystinuria [89], rheumatoid arthritis [90-92], systemic sclerosis [93], primary bdiary cirrhosis [94], heavy metal poisoning due to lead [95], cadmium [%], and mercury [97], and hyperviscosity syndrome [99]. In rheumatoid arthritis, D-peni-cdlamine has been widely accepted as an effective second line treatment. Despite of its effectiveness, it causes many adverse effects, such as skin rashes [99,100], taste abnormalities [100,101], hepatic dysfunction [102-104], gastrointestinal toxiciiy [99,105], proteinuria [100,106], hematuria [107, 108], thrombocytopenia [92, 109], aplastic anemia [110], lupus-like syndrome [111, 112], Goodpasture s-tike pulmonary renal syndrome [113-115], vasculitis [116,117], myasthenia gravis [118-122], polymyositis [123, 124], and dermatomyositis [125]. 

Hyperviscosity of blood ie,j3oVcytl mja yera) Hypereoaguability of blood S... 

Pentoxifylline improves deformability of erythrocytes by increasing cellular ATP concentration via a membrane-metabolizing action, which in tnm rednces the aggregation of erythrocytes and local hyperviscosity. It stimulates prostacyclin formation and release, and inhibits phosphodiesterase degradation of platelet cAMP. The increase of cAMP levels decreases the synthesis of thromboxane A2, and the net resnlt is rednced platelet aggregation. It... 

Thin, dry smears of the semen should be made for a morphologic classification of the sperm forms and should be stained with either hematoxalin or the more difficult, yet more precise, Papanicolaou technique. Also of importance to record is obvious sperm agglutination, pyosper-mia, delayed liquifaction (greater than 30 minutes), and hyperviscosity. In addition, pH, using nitrazine paper, should be determined. 

Hematologic Hematological adverse events are rare they include hemolysis, venous thrombosis and stroke, hyperviscosity, leukopenia, and anemia [1 , 54", 57"]. 

Blood viscosity Hyperviscosity and hyperviscosaemia, Dintenfass L, Kluwer, 1985, 148.75 Clinical blood Rheology, Lowe G.D, CRC Pr, 1988, 137 Flow properties of blood other biological systems (meeting), Copley A, Franklin (Pergamon Press Reprint), 205... 

A condition in which there are excess macroglobulins in the blood. The high levels of large molecular weight proteins lead to sluggish blood flow and this, in turn, can result in thrombosis in the small blood vessels. Retinal vein thrombosis, cerebral thrombosis and peripheral gangrene (hyperviscosity syndrome) are features. 

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