Photopheresis extracorporeal

Author:Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

ECP; Extracorporeal photopheresis

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DefinitionThis section has been translated automatically.

PUVA therapy of leukocytes in an extracorporeal circulation (leukapheresis-based immunomodulatory therapy). Direct cytotoxic/cytostatic effect of methoxsalen by means of targeted irradiation, especially of the lymphocytes circulating in the blood, with UVA (365 nm; so-called photoadduction of methoxsalen to the DNA helix). It is assumed that the reinfusion of the surface-labelled lymphocytes with Methoxsalen stimulates the immune system against antigenic determinants of the autoreactive or malignant cell clones.

General definitionThis section has been translated automatically.

Duration of treatment of extracorporeal photopheresis (ECP) approx. 3-4 hours. The extracorporeal circulation contains 540 ml leukocyte-rich blood plasma, cumulative GD UVA approx. 2 J/cm2. Performance of a cycle on 2 consecutive days. Cycle intervals depend on the disease and the success of therapy (see Table 1).

EffectsThis section has been translated automatically.

The mechanism of action of ECP is not yet fully understood. ECP is considered an immunomodulatory therapy with a shift in the cytokine expression pattern from a pro-inflammatory to an anti-inflammatory profile. ECP causes an increase in the peripheral CD3/NK population and a decrease in the granulocyte-elastase-alpha 1-protease inhibitor complex as well as a decrease in sulfidoleukotrienes and stimulation of the synthesis of TNF-α. Furthermore, apoptosis of mononuclear cells and induction of CD4/CD25-positive T-regulatory cells has been demonstrated.

IndicationThis section has been translated automatically.

Extracorporeal photopheresis has so far only been approved for cutaneous T-cell lymphoma! See table 1.

ContraindicationThis section has been translated automatically.

Insufficient peripheral access, so that the minimum flow rate of 7 ml/min is not granted. Here, permanent subcutaneously implantable hemodialysis catheters with a maximum length of 48 cm (Covidien Healthcare Germany [formerly Tyco Healthcare, Germany]) can guarantee sufficient flow volumes.

LiteratureThis section has been translated automatically.

Apisarnthanarax N (2003) Treatment of cutaneous T cell lymphoma: current status and future directions. At J Clin Dermatol 3: 193-215
Ferenczi K (2003) Monitoring the decrease of circulating malignant T cells in cutaneous T-cell lymphoma during photopheresis and interferon therapy. Arch Dermatol 139: 909-913
Heald P et al (1992) Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy. J Am Acad Dermatol 27: 427-33
Huyn J et al (2006) Extracorporeal photochemotherapy - mechanism of action and clinical applications - focus on transplantation medicine. Act Dermatol 32: 436-439
Liang G et al (1992) Pemphigus vulgaris treated with photopheresis. J Am Acad Dermatol 26: 779-80
Macheiner W (2003) Sezary syndrome and seronegative polyarthritis: treatment with extracorporeal photochemotherapy. J Am Acad Dermatol 48: 220-226
Messina C et al (2003) Extracorporeal photochemotherapy for paediatric patients with graft-versus-host disease after haematopoietic stem cell transplantation. Br J Haematol 122: 118-127
Rook AH et al (1992) Treatment of systemic sclerosis with extracorporeal photochemotherapy. Arch Dermatol 128: 337-342
Suchin KR (2002) Treatment of cutaneous T-cell lymphoma with combined immunomodulatory therapy: a 14-year experience at a single institution. Arch Dermatol 138: 1054-1060
Ständer H et al (2007) Extracorporeal photopheresis with permanently implanted subcutaneous atrial catheters. JAAD 5: 1112-1119
Vonderheid EC et al (1990) Extracorporeal photopheresis in psoriasis vulgaris: clinical and immunologic observations. J Am Acad Dermatol 23: 703-12
Wilfert H et al (1989) Treatment of psoriatic arthritis by extracorporeal photochemotherapy. Br J Dermatol 122: 225-32
Wollina U et al (1999) Progressive systemic sclerosis - treatment results of extracorporeal photopheresis. dermatologist 50: 637-642

TablesThis section has been translated automatically.

Indications for extracorporeal photopheresis

	Disease	Therapy modality	Remarks
Secured indication	Leukemic cutaneous T-cell lymphoma (without stage III = tumor stage)	Cycle intervals every 14 days for 6 months, then extension to monthly intervals, end of therapy after 2 years (a total of 30 cycles with 2 photophereses each)	Ideal response rates in erythroderma and Sézary syndrome up to 70% (here possibly in combination with interferon alfa s.c.)
	Progressive systemic scleroderma (PSS)	Cycle intervals at monthly intervals over 1 year, with positive effect slow extension of intervals to 3-month intervals, maintenance therapy 2-4 cycles per year	Ideal response especially in the acral ascending type, possibly in combination with low-dose glucocorticoids (2.5-7.5 mg/day prednisolone equivalent)
	pemphigus vulgaris	like PSS
	Severe atopic eczema	like CTCL, possibly maintenance therapy
	Dermatomyositis (not as paraneoplasia)	like PSS
	Severe psoriasis vulgaris/arthopathica	like PSS
	Rheumatoid arthritis	like PSS
	graft-vs-host disease	like PSS

Experimental approach	Heart Transplantation	individually, possibly several times a week	Significant reduction of the mortality rate in patients with allogeneic stem cell transplantation
	multiple sclerosis
	Scleromyxedema (Arndt-Gottron)

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer