Six-year Follow-up of a Patient with Multiple Angiomatosis Involving Skeleton, Thoracic and Abdominal Cavities and the Gut Wall

Česká verze

Authors: Z. Adam ¹; M. Matýšková ¹; M. Tomíška ¹; Z. Řehák ²; R. Koukalová ²; L. Křikavová ³; L. Pour ¹; M. Krejčí ¹; P. Szturz ¹; L. Zahradová ¹; M. Mechl ³; M. Moulis ⁴; J. Vaníček ⁵; Č. Neuman ⁶; M. Navrátil ¹; K. Veselý ⁷; R. Hájek ¹; J. Mayer ¹
Authors place of work: Interní hematoonkologická klinika, LF MU a FN Brno ¹; PET CT oddělení, Masarykův onkologický ústav, Brno ²; Radiologická klinika, LF MU a FN Brno ³; Ústav patologické anatomie, LF MU a FN Brno ⁴; Klinika zobrazovacích metod, LF MU a FN u sv. Anny Brno ⁵; Chirurgická klinika, LF MU a FN Brno ⁶; I. patologicko-anatomický ústav, LF MU a FN u sv. Anny Brno ⁷
Published in the journal: Klin Onkol 2012; 25(1): 47-62
Category: Kazuistiky

Summary

Backgrounds:
Multiple angiomatosis is a rare disease causing angiomatous lesions in multiple organs and tissues with a risk of life-threatening haemorrhage.

Observation:
A young man was diagnosed with multiple angiomatosis at the age of 28 after two years of back and abdominal pain. Laparotomy revealed multiple spongy lesions mostly within the retroperitoneal space. Also, an involvement of the gut wall, bones and mediastinum was evident. After 6 years of treatment, the disease has been stabilized. Bone pain ceased with a significant contribution of zoledronate. Using CT and MR imaging, the effectiveness of antiangiogenic drugs was evaluated. Furthermore, treatment response was evaluated using laboratory values for coagulation and blood count, as angiomatous proliferation is known to be associated with disseminated intravascular coagulation and anaemia.

Results:
Baseline laboratory examination revealed elevated D-dimer (more than 20 µg/mL), low fibrinogen (1.4 g/L), and the presence of fibrin monomers. After treatment with 6 mil. IU of interferon-alpha thrice weekly, there was only partial improvement in D-dimer (17.2 µg/mL) and fibrinogen (1.5 g/L) concentrations but fibrin monomers remained positive. After thalidomide (100 mg daily), D-dimer decreased to 6.1 µg/mL and fibrinogen levels increased to 1.9 g/L with the disappearance of fibrin monomers. CT scanning showed significant regression of angiomatous lesions. Progressive neuropathy was the reason to lower the dose of thalidomide by half and this caused D-dimer to rise again. Switching to lenalidomide 10 mg daily led to an increase in D-dimer to 10.8 µg/mL and decrease in haemoglobin concentration to 124 g/L. Fibrin monomers became positive again. Combined therapy with thalidomide (50 mg/day) and lenalidomide (10 mg days 1–21 in 28-day cycles) has led to stabilisation of the disease. Median concentration of haemoglobin increased to 131 (84–141) g/l. The median of D-dimer decreased to 9.3 (8.0–17) µg/mL.

Conclusion:
Thalidomide in the dose of 100 mg daily led to better stabilisation of the disease than interferon-alpha. However, lowering the dose because of adverse effects failed to be effective sufficiently. Lenalidomide 10 mg daily was well-tolerated but insufficient to improve D-dimer and haemoglobin concentrations. Therefore, for further treatment we have decided to use the combination of lenalidomide and thalidomide in doses of 10 mg and 50 mg, respectively because both drugs have desirable antiangiogenic activities with different adverse effect profiles. On this therapy, the patient’s disease has been stable for 9 months.

Key words:
angiomatosis – zoledronate – thalidomide – interferon alpha – lenalidomide – disseminated intravascular coagulation

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
29. 11. 2011

Accepted:
3. 7. 2011

Zdroje

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