Hematology

TPE is simply the replacement of a patient's plasma by normal donor plasma or a suitable substitute,'

Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic Thrombocytopenic Purpura (TTP) a a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure.

TTP is a specific form of thrombotic microangiopathy characterized by the association of a microangiopathic hemolytic anemia, a consumptive thrombocytopenia, and organ failure of variable severity, where typically the brain, the digestive tract a. the heart are involved. UP can be distinguished from other thrombotic microangiopathies by a severe deficiency (< 10%) in the plasma enzyme ADAMTS13."

TTP is more frequent during adulthood, affecting 2 to 4 people per million per year, with a predominance for females (3 to 1 female to male ratio).

TPE is recommended as the first-line treatment of immune-m.iated UP - iTTP (Category I, Grade 1A).

Complications of TPE for iTTP

Catheter-related complications

• Catheter.related bleeding
  • Hemothorax
  • Retroperitoneal hemorrhage
  • Insertion site hemorrhage
• Catheter.related thrombosis
• Catheter.related local or systemic infection
• Catheter dysfunction

Multiple Myeloma (MM)

TPE is traditionally performed I hyperviscosity, neuropathy and to mitigate renal injury in the setting of high clonal free light chain burden in patients with MM with unknown clinical benefit."

There was a significant increase in the proportion of patients receiving TPE from 1% of MM hospitalizations in TPE procedure.related complications

• Hypotension
• Arrhythmia
• Hypocalcemia
• Hypokalemia
• Filter clotting (filtration)
• Reactions to plasma
  • Anaphylaxis
  • Serum sickness
  • Transfusion.related acute lung injury

1993 to 1999 to 2% in 2008 to 2015 (1. for trend <.001). The overall inhospital mortaliry on the other hand, has decreased significantly during the time period (P for trend <.001).

The median (interquartile range) charges for hospitalized MM patients for TPE increased significantly over time (P for trend <.0001). The median (interquartile range) length of hospitalized MM patients who received TPE remained unchanged over time (ill (6-19) days in 19931999 vs 10 (616) in 2000-2007 vs 12 (7-18)in 2008-2015, P for trend .18)

In hospital mortality of hospitalized MM patients who received TPE decreased from 1993 to 2015 (17.5% (SE 0.3) in 1993-1999, 9.5% (0.2) in 2000-2007 and 8.9% (0.2) in 2008 to 2015) (P for trend <.001). The role of TPE in MM is limited to conditions such as hyperviscosity syndrome.

There has been a substantial increase in the use and associated cost of TPE in hospitalized MM patients.

Acquired Factor VIII Positive Hemophilia

In the general population, Acquired Hemophilia (AH) is uncommon, with an estimated frequency of one case per million. AH is linked to unexpected and significant bleeding, which can be life-threatening. Factor substitutiort immunosuppression (e.g., with corticosteroids and cyclophosphamide), high-dose IVIG, plasma exchange (PE), and immunoadsorption (IA) are all options for treatment. Immunosuppression combined with PE may be useful in individuals who fail to respond to other treatments by eliminating pathologic autoantibodies and replacing fluid with fresh frozen plasma or albumin.. An 82-year-old male patient was transferred to the hospital after the diagnosis of AH. Anti-nuclear antibodies, extractable nuclear antigens, and the Coombs Test were all negative in the immunological serum parameters. Treatment with high-dose intravenous (IV) corticosteroids (500 mg) was started right away, along with TPE Consequently, PE was performed with replacement by fresh frozen plasma (10 units per session). Of note, after 36 sessions of PE, FVIII was stable (>50%) and further normalized (>90%) after 1 g IV cyclophosphamide was given on day 28. No FVIII inhibitor could then be detected.

On treating patient with plasma exchange, corticosteroids, cyclophosphamide and mycophenolate mofetil CM MF), an immediate decline in titer i nhi bitors can be obtained as a result of plasma replacement, and prog ress can be sustained with long-term moderate immunosuppression with MMF preced. by one injection of IV cyclophosphamide.

Severe ABO incompatible

TPE is performed to remove ABO antibodies and permit ABO-incompatible (ABO-I) kidney transplants, but there is only limited research within this area and a lack of standardized protocols for TPE.. It is the naturally occurring antibodies that are produced against ABO antigens that are not present in one's own tissues that are key mediators of Antibody-mediated Rejection (AMR) and prevent renal transplantation across ABO barriers. The ABO blood group transplantation barrier was revered as insurmountable until recently due to initially poor experiences crossing the barrier.. ABO-I kidney transplantation is currently an ASFA Category II indication for TPE. TPE treatment plan is based on the anti-A and anti-B titers with the goal of achieving ABO titer at the AHG phase of 16 or less before surgery.

ABO antibody titers are closely monitored after ABO-1 kidney transplantation can be successfully transplantation and it is important for all patients to performed after a preconditioning regimen of TPE/ routinely receive several posttransplant TPE/CMVIg CMVIg. Patients who have high initial ABO antibody titers treatments. Posttransplant TPE therapy appears to be often require more TPE/CMVIg treatments to achieve helpful in preventing rebound of anti-A and anti-B titers therapeutic endpoints.. until tolerance or accommodation occurs.

Pregnancy

TPE is usually not indica. prior to delivery. However, in rare cases, Thrombotic Microangiopathy (TMA, is caused by diseases unrelated to the placenta i.e., UP, atypical Hemolytic Uremic Syndrome (aHUS) or acquired TMA secondary to drugs, antiphospholipid autoantibodies, or viral infections. Pregnancy is estimated to account for the presenting episode of TTP in 10-25% and the risk of relapse of UP in pregnancy is between 60 and 90%. TPE is the first-line treatment and should be started as soon as possible to remove antiADAMTS-13

autoantibodies as well as replenish ADAMTS-13, Therapeutic management of pregnant women can be tailored: TPE is first-line treatment for UP with addition of immunosuppression such as high dose corticosteroids, while for aHUS potent inhibition of complement activation with eculizumab should be considered as an additional therapeutic strategy to TPE in order to overcome the continuous need for TPE and potentially improve renal recovery,