Photopheresis extracorporeal

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

ECP; Extracorporeal photopheresis

Definition
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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 definition
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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).

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Effects
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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.

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

Contraindication
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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.

Literature
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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

Tables
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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)