Acral Ischemia Research Articles

Thrombangitis obliterans: Subpopulationen der Leukozyten und zirkulierende Immunkomplexe

Background: The etiology of thrombangitis obliterans is still unclear. Although cellular infiltration of the vessel wall is known, no studies on peripheral blood mononuclear cells are reported. Therefore, we assessed leucocyte subpopulations and circulating immune complexes in patients with thrombangitis obliterans and a control group of normal people. Patients and methods: 31 patients (40 ± 2 years, 24 male, 7 female) with thrombangitis obliterans were included, based on the following criteria: age of manifestation, acral ischemia in legs and arms, previous thrombophlebitis or phlebitis saltans. Manifestation of atherosclerosis or other vasculitic manifestations were excluded. Leucocyte subpopulations, levels of C-reactive protein (CRP) and circulating immune complexes (CIC) were investigated. An age-matched control group (n = 25) was recruited from voluntary blood donors. Results: Leucocyte counts in the thrombangitis group (mean ± SD: 10839 ± 782/nl) were significantly different from the control group (6205 ± 414/nl, p < 0.0001). The same was true for absolute counts of granulocytes, monocytes and lymphocytes. The results were independent from CRP, which was elevated only in 6 patients. Relative counts of naive helper T-cells were significantly lower in the patient group. HLA-DR expression on B-cells was lower on the patients’ lymphocytes. The concentrations of IgA, IgG and IgM in CIC were higher in the thrombangitis patients compared to the control group. C1q-binding capacity and phosphatidylserine antibodies showed no differences. Conclusions: Patients suffering from thrombangitis obliterans show alterations of leucocyte counts and their subpopulations as well as alterations of the humoral (IgCIC) immune system.

Capillary Microscopy Is a Diagnostic Aid in Patients with Acral Ischemia

The diagnosis of peripheral ischemic diseases is a challenge to the vascular specialist, since discrimination of the different entities on clinical grounds is not always clear. Capillary microscopy is a noninvasive diagnostic technique that can visualize in particular the morphology and perfusion of the digital capillaries. The authors investigated all 522 patients who were referred to the vascular laboratory over a six-year period, in an attempt to obtain more certainty about the diagnosis of their acral ischemic complaints. Patients with clinically manifest Raynaud's phenomenon and control subjects were compared to obtain differentiation criteria. Thus, capillary microscopy scored a sensitivity of 67%, a specificity of 84%, and an accuracy of 81% to detect Raynaud's phenomenon. A disturbed capillary morphology was virtually pathognomonic in Raynaud's disease secondary to systemic disorders (specificity 100%); the accuracy was but 74%. In 69% of the patients with clinically atypical acral ischemia, capillary microscopy yielded a diagnosis. Digital blood pressure measurements, however, appeared of no use in the diagnosis of Raynaud's phenomenon. Hence, capillary microscopy appears to be a useful aid in the diagnosis of acral ischemic syndromes.

Acral Lividosis—A Sign of Myeloproliferative Diseases

Acral ischemia with lividity is a well-described dermatologic sign in the myeloproliferative diseases polycythemia vera and essential thrombocythemia. It has not previously been reported as a sign of chronic myelogenous leukemia (CML). We suggest the term acral lividosis to describe this clinical entity in patients with any myeloproliferative disease. We propose that the pathophysiology of acral lividosis in CML involves occlusion of small blood vessels of the skin by large, nondeformable myeloblasts, a process that has been shown histologically to occur in other organs in patients with CML. This process, called leukostasis, occurs in patients with CML who have over 50.0 X 10(9)/L (50,000/mm3) circulating myeloblasts. Patients manifest cardiorespiratory and central nervous system compromise, a clinical constellation known as the hyperleukocytosis syndrome. Acral lividosis occurred in a patient with CML in whom nearly every organ demonstrated leukostasis on autopsy.