Scientific corner

GS2-03 Japanese apheresis guidelines for the management and treatment of generalized pustular psoriasis, pustulosis palmoplantaris and psoriasis arthropathica

Miho Hatanaka, Yuko Higashi, Takuro Kanekura

poster at ISFA 2019 pag 104

Generalized pustular psoriasis (GPP) is a rare disease characterized by recurrent fever and
systemic flushing accompanied by extensive sterile pustules. Treatments of GPP are usually
topical corticosteroids, activated vitamin D3 ointment, ultraviolet light (UV) therapy, and
oral administration of etretinate, cyclosporine, or methotrexate. Recently, biologics such as
TNF- α; inhibitors, anti-IL-17- and anti-IL-23 antibodies are used. Pustulosis palmoplantaris
(PPP) is a chronic recurrent disorder of the palms and soles characterized by sterile intradermal
pustules. PPP often accompanies joint symptoms. In some instances, PPP is associated with
a focus of infection somewhere in the body; elimination of the infection sometimes improve
symptom. Some treatments of GPP are used for PPP. Psoriatic arthritis (PsA) is a disease
characterized by skin and nail psoriasis together with widespread musculoskeletal inflammation
such as peripheral joint disease, axial joint disease, enthesitis, and dactylitis. Treatment of
PsA is oral administration of NSAID’s, cyclosporine, methotrexate and phosphodiesterase 4
inhibitors for mild to moderate cases. Biologics; TNF- αinhibitors, anti-IL-17- and anti-IL-23
antibodies; have been approved for severe or advanced cases. Granulocyte/monocyte adsorption
apheresis (GMA) is an extracorporeal therapy designed to remove and suppress the functions
of neutrophils, macrophages and monocytes that accumulate in the inflamed tissue and are
involved in the pahogenesis. GMA may be considered as a safe treatment modality with few
side-effects for GPP, PPP and PsA. The effect and safety of GMA have been reported mostly in
case reports. Although the effect and safety of GMA were demonstrated in a multicenter study.
GMA’s utility is expected based on the mechanism of action.

Scientific corner

LS2-02 Tips for ensuring vascular access and maintaining extracorporeal circulation in pediatric blood purification therapy

Mariko Sawada

poster at ISFA 2019 pag 164-165

Ensuring reliable vascular access (VA) and maintaining stable extracorporeal circulation are the most basic aspects of blood purification therapy (BPT). In children and neonates, specific tips could be helpful for BPT.
VA guidelines were published in 2011 and management methods have been unified. To ensure VA, it is necessary to determine a suitable placement site and catheter size (diameter and length), adjust the catheter tip position, and manage the catheters appropriately. It is common to use dialysis catheters for BPT, placing them in the central and peripheral veins. In neonates, the umbilical vein could also be one of the options, and central venous catheters and peripheral vein catheters could be used for BPT. In order to maintain stable extracorporeal circulation, it is necessary to maintain sufficient intravascular volume and blood pressure, set appropriate blood flow rates, and adjust the type and amount of anticoagulant. In children who cannot cooperate,
sedation management and catheter fixation should be performed to stabilize extracorporeal circulation.
There are also tips specialized for each disease state. In neonates, there is a high risk of intracranial hemorrhage and nafamostat mesylate is often used as an anticoagulant. In addition, it is necessary to increase the dose of anticoagulant or administer it from two places in the circuits. In patients with severe inflammatory bowel diseases, intestinal bleeding continues despite increased clotting function and hypovolemia is common. Heparin and nafamostat mesylate are chosen as anticoagulants. During BPT, monitoring activated clotting time, administering minimal anticoagulants, and administering transfusion and fluid load are useful methods to maintain stable extracorporeal circulation. BPT might be a powerful therapeutic tool for children as well as adults, ensuring reliable VA and maintaining stable extracorporeal circulation.

Scientific corner

LS2-01 Vascular punctures for GMA treatment

Hisashi Matono1), Akiyoshi Okada2), Junya Imada1), Tetsushi Nakahara1),
Atsushi Ono1), Takuma Usuda1), Atsuo Kitano

poster at ISFA 2019 pag 164

We can provide the GMA treatment by venepuncture without VAC detaining you like other blood purification therapy. The puncture by the peripheral vein puncture is easy, but, on the other hand, it often becomes difficult to secure blood flow necessary for extracorporeal circulation enough and is one of the trouble factors of the GMA treatment We use an echo to
evade a puncture trouble in our institution. We introduce the approach this time.
Approach method The site of puncture uses brachial veins basically. There is little valve of vein as a reason and chooses the blood stream because securing of blood flow with a little meandering is relatively easy. However, the depth from skin may be deepened as compared with the erasion blood vessel such as radius cutaneous veins, and the like, too, and attention is necessary. We often perform GMA in outpatient department at this hospital, and order enters the new induction when a chief physician judged induction from an encounter. Therefore it is difficult to obtain the patients information beforehand and a blood vessel echo uses site of puncture after the patients admission and is determined and performs the puncture in echo guides, if necessary. Also, the judgments such as the dehydration, and the like are possible by using an echo, and there is the
merit that they receive instructions such as the transfusion load, and the like in what we report to a chief physician, and can dissolve puncture difficulty.
Conclusion One of the troubles of the GMA treatment has securing of access. If extracorporeal circulation is possible without poor blood removal, we can provide GMA treatment more effectively. We regard the echo inflection of that purpose as a required device on providing GMA treatment.

Scientific corner

The selective therapeutic apheresis procedures

The selective therapeutic apheresis procedures.

J. Clin. Apheresis 28:20–29, 2013  doi:10.1002/jca.21265 

Selective apheresis procedures have been developed to target specific molecules, antibodies, or cellular elements
in a variety of diseases. The advantage of the selective apheresis procedures over conventional therapeutic plasmapheresis is preservation of other essential plasma components such as albumin, immunoglobulins, and clotting
factors. These procedures are more commonly employed in Europe and Japan, and few are available in the
USA. Apheresis procedures discussed in this review include the various technologies available for low-density
lipoprotein (LDL) apheresis, double filtration plasmapheresis (DFPP), cryofiltration, immunoadsorption procedures, adsorption resins that process plasma, extracorporeal photopheresis, and leukocyte apheresis.

Scientific corner

The present status and the recent development of the treatment for inflammatory bowel diseases: desirable effect of extracorporeal immunomodulation

Masakazu Takazoe 1Torao TanakaKenji KondoToshiki IchimoriToshio Shinoda Ther Apher 2002 Aug;6(4):305-11. doi: 10.1046/j.1526-0968.2002.00445.x.

The immunological and genetic pathogeneses of inflammatory bowel disease (IBD) have been well elucidated in the recent years. The pharmacologic treatment of IBDs accordingly becomes to focus upon the individual pathologic step (targeting therapy), whereas the therapeutic action is not yet a pinpoint one. It has been known recently that new drugs such as biological immunomodulating agents and anti-inflammatory cytokines have better short-term effects in some respects than the conventional drugs, and they might alter the treatment strategy of IBDs in the near future. The limitation of pharmacologic treatments mainly results from adverse effects of the drugs, i.e. infection susceptibility, oncogenesis, teratogenesis and so forth. The extracorporeal therapy such as leukocytapheresis and photopheresis is reportedly effective for IBDs probably through immunomodulation such as decrease in circulating activated T-lymphocytes and activated granulocytes that play a central role in the pathogenesis of IBD. It can be said that these extracorporeal treatment methods have advantage of rapid action and lack of serious adverse effects to drug therapy.

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