Nursing Management of GVHD

GVHD의 정의/의미 정의 의미 동종조혈모세포이식 생착 후 발생하는 합병증 이식편(graft)이 숙주(host)의 장기를 공격 동종조혈모세포이식 생착 후 발생하는 합병증 이식편(graft)이 숙주(host)의 장기를 공격 각종 장기의 손상 / 기능 장애 초래 의미 생착 후 발생하는 가장 흔한 합병증 이식 후 morbidity/mortality에 큰 영향을 미침 이식 환자의 삶의 질(quality of life)과 밀접한 관계 혈액암에 대한 이식에서는 재발을 감소시키는 효과

Definition of aGVHD and cGVHD Acute GVHD 과거의 정의 : 이식 후 100일 이내 발생 최근의 정의 : 이식 초기에 피부발진/ 설사/ 간기능 장애 등으로 나 타나는 GVHD Chronic GVHD 과거의 정의 : 이식 후 100일 이후 발생 최근의 정의 : 다양한 장기에서 나타나는 “자가면역질환” 양상의 보이는 증후군

Definition of aGVHD and cGVHD 과거의 정의 D-7~D100 D100~ 최근의 정의 D-7~D100 D100~ Acute Chronic 이 식 Classic Ac. Persistent, recurrent, late-onset Acute Classic Chronic Overlap syndrome

이식 전처치(방사선/항암제)에 의한 수혜자 조직 손상 발생기전 - aGVHD 이식 전처치(방사선/항암제)에 의한 수혜자 조직 손상 염증과 관련된 싸이토카인 분비 공여자 T세포 활성화/증식/ 분화 면역기전에 의한 장점막/간/피부의 조직 손상

발생기전 - cGVHD CMV 감염증 T 림프구의 이상 그 외, 자가면역질환의 발생 기전들이 기여 : 확실히 밝혀진 바는 없음. T 림프구의 이상 : T 림프구는 세포 면역의 활성/억제를 조절하는 기능을 담당 · 숙주의 조직에 대한 공격을 유도하는 T림프구의 증식 · T 림프구의 작용 조절 기전의 상실 B 림프구의 이상 : B 림프구는 체액 면역(항체 형성)을 담당 싸이토카인의 불균형 : 싸이토카인은 면역 반응에서 염증 유발을 유도 CMV 감염증 그 외, 자가면역질환의 발생 기전들이 기여

Risk factors Recipient Donor 이식과 관련된 특성 확립된 사항 고령 만성 골수성 백혈병 재생불량성 빈혈 여성기증자(특히 다산부) → 남성 수혜자 조직적합항원 불일치 또는 비혈연간 이식 말초조혈모세포 사용 DLI(공여자림프구주입술) T 세포가 다량 포함된 경우 Acute GVHD 논란의 여지가 있는 사항 CMV 혈청 양성 CMV 활성화 비장절제 기증자와 수혜자간의 인종차이 제대혈이식(위험도가 낮아짐) acute GVHD 예방을 위해 steroid를 사용한 경우 말초조혈모세포 사용시 CD34+ 세포수가 많은 경우 GVHD 예방에 MTX를 사용 하지 않은 경우 ·Conditioning regimen의 종루(BuCy vs. TBI/Cy) ·aGVHD 예방을 위해 사용한 면역억제제의 종류(CsA vs. tacrolimus) ·MTX의 투여 횟수 (3 vs. 4)

aGVHD – Clinical manifestations Organ / Site Clinical manifestations Skin Maculo-papular rash, often involving the palms and soles Pruritic and / or painful In more severe cases, Bullae can form and surface areas can desquamate Liver Elevated liver enzymes(Bilirubin, alkaline phosphatase) RUQ pain Hepatomegaly Jaundice GI tract Nausea & vomitting and green watery diarrhea Severe abdominal pain, bloody diarrhea Anorexia Mucositis Paralytic ileus

Clinical Staging of aGVHD Stage Skin Liver GI tract +(1) Maculopapular rash < 25% body sulface Bilirubin 2~3mg/dl Diarrhea 500~1000ml/day ++(2) 25~50% body sulface 3~6mg/dl 1000~1500ml/day +++(3) Generalized Erythroderma 6~15mg/dl > 1500ml/day ++++(4) Erythroderma with bullous formation and desquamation > 15mg/dl Cramp, ileus

Functional Grading of aGVHD Grade Skin Liver GI tract 0 (none) Ⅰ(mild) + or ++ Ⅱ(moderate) + to +++, or +, or + Ⅲ(severe) ++ to +++, or ++ to +++ Ⅳ(life-threatening) ++ to ++++, or ++ to ++++

cGVHD GVHD Relapse

cGVHD - Classification acute GVHD가 그대로 진행 Poor prognosis Progressive Acute GVHD 치료 후 다시 발생 Quiescent 100일 이후 처음 생김 excellent prognosis De novo

Criteria for Diagnosis of cGVHD Limited cGVHD Either or both : 1. Localized skin involvement 2. Hepatic dysfunction because of cGVHD Extended cGVHD 1. Generalized skin involvement Or; 2. Localized skin involvement and/or hepatic dysfunction because of cGVHD Plus; a. Liver histology showing chronic aggressive hepatitis, bridging necrosis or cirrhosis b. Involvement of eye : Schimer`s test with less than 5mm wetting c. Involvement of minor salivary glands or oral mucosa demonstrated on labia biopsy specimen d. Involvement of any other target organ

Signs and symptoms of cGVHD Organ/site “Diagnostic” “Distinctive” Other Both acute and chronic Skin Poikiloderma (피부위축,변색) Lichen planus-like feature(반사성 적자색 구진) Depigmentation Erythema, Maculopapular rash, pruritus Nails Dystropy : ridging, splitting, brittleness Scalp Alopecia Mouth Lichen planus(그물모양/레이스 모양병변) Xerostomia Mucositis Eyes Keratoconjunctivitis sicca(건조성 각결막염) Photophobia, blepharitis(안검염, 눈꺼풀테염증) Genitalia Lichen planus

Signs and symptoms of cGVHD Organ/site “Diagnostic” “Distinctive” Other Both acute and chronic GI tract Strictures of esophagus(식도협착) Exocrine pancreatic insufficiency(췌장의 효소 분비 이상) Anorexia, weight loss Liver Bilirubin or alkaline phosphate >2x upper limit of normal Lung Bronchiolitis obliterans(폐쇄성 기관지염) Muscles, fascia, Joints Fascitis, joint contractures Myositis Cramps, arthralgias Hematopoietic and immune Thrombocytopenia eosinophilia lymphopenia Ascites, pericardial or pleural effusions

aGVHD – Nursing Management Skin Macular papular rash, erythroderma Cleanse skin with mild antimicrobial soap and rinse well Prevent dryness with frequent lubrication Prevent injury to skin Bullous formation and desquamation Apply lubrication and antimicrobial creams/ointments Apply hydrogel dressings and secure with gauze wraps Allow debridment of loosened skin during dressing changes and baths Monitor response to pain management Evaluate need for specialized beds

aGVHD – Nursing Management GI tract Monitor character and guaiac of stool and emesis Strictly monitor intake and output Take weights daily Evaluate oral / enteral nutrition and fluid intake Maintain NPO diet when indicated Administer parenteral nutrition Administer analgesics for severe pain caused by cramping Monitor response to antiemetics Maintain meticulous perinanal care with cleansing and protective lubricants Maintain meticulous oral hygiene with normal saline mouth rinses

aGVHD – Nursing Management Liver Monitor liver function tests and coagulation studies Assess color of skin / sclera Monitor for signs of bleeding Maintain fluid and electrolyte balance / restriction Monitor changed in renal function Take daily weight and abdominal girth measurements Assess neurologic status

cGVHD – Nursing Management Organ/site Clinical Manifestations Nursing management Skin Scleroderma Poikiloderma Ulcerations Decreased range of motion Lichen planus lesions Erythematous rash Hyper – or hypopigmentation Encourage skin cleansing with a nonirritating soap Teach range-of-motion exercises Prevent injury Provide lubrication Liver Elevated liver enzymes (Bilirubin, alkaline phosphatase, gamma glutamyl transferase) Jaundice in advanced situations Change in neurologic status in advanced situations Monitor LFTs and coagulation studies Administer antipruritics or antihistamines Monitor skin / sclerae Monitor neuro status

cGVHD – Nursing Management Organ/site Clinical Manifestations Nursing management Mucous Membranes Lichenoid changes Vaginal dryness Erythema Ulceration Xerostomia Provide and administer lip and oral lubricants Provide sexual counseling Monitor complication to oral hygiene regimen Eyes Keratoconjunctivitis Dry eyes “Gritty” feeling Pain Photophobia Monitor Schimer`s results Consult ophthalmology for evaluation Provide lubrication Prevent comeal abrasion

cGVHD – Nursing Management Organ/site Clinical Manifestations Nursing management GI tract Abdominal pain or cramping Dysphasia Malabsorption/malnutrition Diarrhea Evaluate nutritional and fluid intake Administer pain medication Monitor weight Monitor I & O Provide nutritional IV supplementation as needed Adjust diet in compensating for dysphagia Pulmonary Dyspnea on exertion Diminished breath sounds and wheezing Fatigue and weakness Administer immunosuppressive agents Monitor for signs and symptoms of hypoxemia Monitor response to oxygen or respiratory treatment

cGVHD – Nursing Management Organ/site Clinical Manifestations Nursing management Hematologic Easy bruising Symptoms of bleeding Thrombocytopenia Monitor response to platelet transfusions Prevent injury or trauma