Angioendotheliomatosis Workup

Updated: May 14, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

Peripheral blood smear and indices and serum chemistry results are often unremarkable. As for reactive angioendotheliomatosis, the results depend on the underlying disease. The following findings could be observed:

  • Anemia

  • Elevated erythrocyte sedimentation rate

  • Low platelet count

  • Leukocytosis/leukopenia

  • Elevated lactate dehydrogenase (LDH) levels

  • Abnormalities in cold-reactive proteins are sometimes observed (ie, circulating cryoproteins, elevated cryofibrinogen levels).

  • Increased alkaline phosphatase levels

  • Increased aspartate and alanine aminotransferase levels

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Imaging Studies

In reactive angioendotheliomatosis, echocardiograms, electrocardiograms, and chest radiographs can reveal changes related to the underlying disease (eg, in subacute bacterial endocarditis [SBE], tuberculosis, chronic infection, autoimmune disease).

In diffuse dermal angiomatosis, angiography or Doppler ultrasonography can show stenotic changes of the vessels.

In malignant angioendotheliomatosis, cranial computed tomography and magnetic resonance imaging can frequently demonstrate parenchymal hypodensity consistent with cerebral infarction or a degenerative process.

In bone technetium scintigraphy, results can be normal because bone marrow is typically spared.

Chest radiographs often do not reveal any abnormalities.

Ultrasonography of the abdomen and the pelvis may demonstrate hepatomegaly and splenomegaly; however, no evidence of lymph node involvement exists in the vast majority of patients.

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Procedures

Bone marrow biopsy can be totally normal or demonstrate only benign reactive hyperplasia or, in some cases, a malignant lymphoid proliferation compatible with malignant angioendotheliomatosis. [32]

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Histologic Findings

Dilated dermal and upper subcutaneous fat blood vessels filled with cells and fibrin thrombi may be observed, with occlusion sometimes being evident. In the reactive form, the cells filling the vessels vary in size from small to large, are bland with open chromatin, and contain inconspicuous nucleoli.

Immunohistochemistry is considered the definitive test used to differentiate reactive angioendotheliomatosis from intravascular lymphoma. In reactive variant, immunocytochemistry of intravascular cells demonstrates positivity for Ulex europaeus I lectin and factor VIII–associated antigen (CD31 and CD34), thus the supporting endothelial origin of the cells. Proliferation of pericytic myoepithelial cells is sometimes noted within and around affected blood vessels. [33]

In the malignant form, a proliferation of atypical, hyperchromatic lymphoid cells with numerous mitotic figures predominates. On immunocytochemistry, cells are positive for lymphocyte markers, such as leukocyte common antigen (CLA, CD45RB), and B-cell (CD20, CD45RA, CD79a) and T-cell (CD1, CD3, CD4, CD8, CD43, CD45RO) markers. Most vessels are surrounded by a perivascular infiltrate containing a large number of plasma cells.

In the histiocytic variant, intraluminal proliferation of cell-bearing histiocyte markers, such as Mac387 and KP1 (CD68), can be observed.

In diffuse dermal angiomatosis, lesions are full of diffuse, extravascular proliferations of endothelial cells between collagen bundles of the reticular dermis, with only minimal intravascular proliferation of those cells. Immunoperoxidase studies using CD31 and CD34 markers highlighted the vessels, confirming the vascular nature of the disease.

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