Plasmaphresis & IVIG

Plasmaphresis

血漿置換術(PE) 、血漿雙重過濾術（DFPP）

當我們需要移除致病的免疫球蛋白和發炎物質時：

   1)紅斑性狼瘡（SLE）

   2) 重症肌無力（Myasthenia gravis)

   3) 血栓性血小板低下性紫斑症（TTP)

   4)腎絲球腎炎（RPGN, goodpasture’s syndrome）

   5)格林-巴利綜合征（Guillain barre syndrome）

當我們想移除代謝廢物時：

   1)重症肝炎,

   2)術後肝衰竭,

   3)急性肝衰竭,

   4)原發性膽汁性肝硬化

   5)移除其他代謝廢物

當我們想降低血中的黏稠度時：

  1) 多發性骨髓瘤 (Multiple myeloma)

  2) 高脂血症 (Hpertriglycermia)

疾病名稱	證實治療有效的疾病
腎臟疾病	Goodpasture’s syndrome （肺出血腎炎症候群） 紅斑性狼瘡腎炎
神經系統疾病	重症肌無力 多發性神經根炎 慢性發炎脫髓鞘 多發性神經病變 Refsum disease Guilain – Barre syndrome
血液疾病	紫斑症(輸血，血栓性血小板減少引起) Sickle cell disease 凝血因子抑制疾患
免疫系統疾病	紅斑性狼瘡 類風濕性關節炎 Cryoglobulinemia
腸骨系統及 皮膚系統	天疱瘡
肝臟疾病	猛爆性肝衰竭

副作用：

1.最常見之死因：心律不整，呼吸窘迫與非心因性肺水腫，其他還有過敏、敗血症 以及肝炎等。

2.輸血共同反應：因血管內容積減少造成的低血壓，半透膜生物不相容引發的過敏反應，另外尚有與血管通路有關的問題，如感染，血栓，血腫，血管破裂或動靜脈交通的形成。

Intravenous immune globulin (IVIG)

IgG的替代治療用藥

Indications：

1.原發性免疫不全症（primary immune deficiency）。

2.骨髓瘤（myeloma）與慢性淋巴癌（chronic lymphatic leukamia）引致嚴重次發性免疫球蛋白缺乏（severe secondary hypogammaglobulinaemia）與復發性感染（recurrent infections）。

3.伴隨復發性感染之先天性免疫不全症（AIDS with recurrent infections）。

4.原發性血小板缺乏性紫斑症【idiopathic thrombocytopenic purpura（ITP）】，有出血危險的成人或小孩ITP患者；或者，手術前用以修正血小板數目。

5.異體骨髓移植（allogeneic bone marrow transplantation）。

6.川崎氏症（Kawasaki Disease）。

Immunodeficiency states

•Primary (congenital/hereditary) immunodeficiencies

•Severe protein loss (eg, through malnutrition or gastrointestinal or renal losses)

•Post-hematopoietic cell transplantation (HCT)

•Chronic lymphocytic leukemia

•Multiple myeloma

• Secondary immunodeficiency induced by biologic therapies

●Infections

•Chronic lymphocytic leukemia (CLL) with recurrent infections requiring intravenous antibiotics or hospitalization

•Chronic parvovirus infection complicated by anemia

•Relapsing or severe Clostridium difficile infection

•Toxic shock syndrome – streptococcal toxic shock syndrome

•Measles postexposure prophylaxis (if the patient is immunocompromised or nonimmune)

•Some enteroviral infections

●Autoimmune/inflammatory conditions

•Immune thrombocytopenia (ITP)

•Neonatal alloimmune thrombocytopenia

•Acquired von Willebrand disease (VWD) in the setting of lymphoproliferative or other immunologic disorders

•Autoimmune hemolytic anemia (AIHA)

•Autoimmune neutropenia

•Acquired factor VIII inhibitor

•Guillain-Barré syndrome

•Kawasaki disease

•Chronic inflammatory demyelinating polyneuropathy (CIDP)

•Multifocal motor neuropathy

•Some human immunodeficiency virus (HIV)-associated neuropathies

●Alloimmune processes

•Post-transfusion purpura

•Platelet alloimmunization with bleeding (as a temporizing measure)

•Antibody-mediated organ transplant rejection

•Hyperhemolytic crisis (a form of delayed hemolytic transfusion reaction) in individuals with sickle cell disease (SCD)

Adverse reaction：

1.IMMEDIATE REACTIONS

Rate-related reactions

- Concurrent infections/phlogistic reactions

- Reactions resembling anaphylaxis

- Management of symptoms

Other transfusion reactions

Anaphylaxis in IgA-deficient patients

2.DELAYED REACTIONS

Thromboembolic events

Central nervous system effects

Renal complications

- Acute kidney injury

- Hyponatremia

Hematologic complications

- Hemolysis

- Neutropenia

3.LATE REACTIONS

Dermatologic reactions

Miscellaneous reactions

Effects on vaccine efficacy

Infectious risks