30 Therapeutic Apheresis: Indications, Efficacy and Complications Flashcards
Category I
Apheresis is an accepted first-line therapy for these disorders.
Category II
Apheresis is an accepted second-line therapy for these disorders.
Category III
Individualize decision making. The optimal role of apheresis has not been conclusively determined in these disorders.
Category IV
Published evidence indicates that apheresis is ineffective or harmful in these disorders. Institutional review board approval is desirable if apheresis is planned.
The term that refers to the removal of plasma from the circulation by manual or automated methods
Plasmapheresis
The term that refers to a therapeutic procedure in which plasmapheresis is combined with replacement of the removed plasma by a substitute colloid fluid, most commonly a mixture of 5% human serum albumin and 0.9 percent saline solution
Plasma exchange
The “sweet spot” for plasma exchange procedures is the processing of between ___________ plasma volumes.
1.0 and 1.5
Class I Indications for TPE
- Catastrophic antiphospholipid syndrome
- Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
- Erythrocytosis: Primary (Polycythemia vera)
- Hereditary hemochromatosis (Erythrocytapheresis)
- Hyperviscosity in monoclonal gammopathies: Symptomatic and as Prophylaxis for rituximab
- Sickle cell disease: Acute stroke and Stroke prophylaxis (RBC Exchange)
- Thrombotic microangiopathy-Drug-associated: Ticlopidine
- TTP
- Complement mediated TMA; Factor H autoantibody
Hyperviscosity in monoclonal gammopathies: It is most common in ________________because of the highly red-cell–aggregating properties of immunoglobulin (Ig) M and, less often, in IgG or IgA myeloma.
Waldenström macroglobulinemia
TRUE OR FALSE
The relationship between monoclonal protein level and serum viscosity is nonlinear; therefore, a relatively small (20%) decrease in plasma protein can affect a major change in viscosity.
TRUE
The relationship between monoclonal protein level and serum viscosity is nonlinear; therefore, a relatively small (20%) decrease in plasma protein can affect a major change in viscosity.
Waldenström macroglobulinemia: - Symptoms typically emerge when serum viscosity rises above ________ relative viscosity units (normal being 1.4-1.8).
4.0 relative viscosity units
Are immunoglobulins or complexes of immunoglobulins that reversibly precipitate when exposed to temperatures below 37°C.
Cryoglobulins
Associations of Types of Cryoglobulinemia
Type I: lymphoproliferative disorders
Type II: hepatitis C
Type III: chronic infections or autoimmune disorders
Types of Cryoglobulinemia
Type I: isolated monoclonal immunoglobulins
Type II: a mixture of immunoglobulins including a monoclonal component that exhibits antibody activity toward polyclonal IgG
Type III:mixed polyclonal immunoglobulins of one or more classes
Results from combination of free light chains with Tamm-Horsfall mucoprotein in the distal nephron and the resultant precipitation of obstructing casts
Myeloma cast nephropathy (“myeloma kidney”)
TRUE OR FALSE
Plasma exchange is currently considered to be part of first-line treatment for myeloma with cast nephropathy, and is an option when renal function does not rapidly improve with chemotherapy.
FALSE
Plasma exchange is not currently considered to be part of first-line treatment for myeloma with cast nephropathy, but may be a reasonable option when renal function does not rapidly improve with chemotherapy.
A medical emergency that presents with microangiopathic hemolytic anemia and thrombocytopenia
Idiopathic thrombotic thrombocytopenic purpura (TTP)
In acquired idiopathic TTP, this enzymatic defect is caused by an autoantibody inhibitor of __________that results in severe deficiency of the enzyme
ADAMTS-13
TPE, using ________ as the colloid exchange fluid, was the only therapy for TTP that has been demonstrated highly effective in a randomized clinical trial.
Human plasma
The chimeric anti-CD20 monoclonal antibody that has been shown to reduce the risk of relapse when administered to patients with TTP who achieve remission with TPE.
Rituximab
A humanized immunoglobulin fragment that prevents interaction of platelets with the A1 domain of von Willebrand factor, appears to shorten the course of TPE required to achieve remission in patients with TTP.
Caplacizumab
A thrombotic microangiopathy with acute oliguric or anuric renal failure, is rarely associated with severe deficiency of ADAMTS-13.
Hemolytic uremic syndrome (HUS)
Shiga toxin–associated HUS does not respond to TPE; atypical HUS (ie, with defects in regulation of the complement system) has shown only limited responses to TPE
Shiga toxin–associated HUS does not respond to TPE; atypical HUS (ie, with defects in regulation of the complement system) has shown only limited responses to TPE and is more appropriately treated with_________________.
Eculizumab
The two most common drugs reported to the FDA as associated with TTP are
Ticlopidine and clopidogrel
Drug-associated TTP
Autoantibodies to ADAMTS-13 are seen in ____________-associated TTP
Ticlopidine-associated TTP
TRUE OR FALSE
Patients with clopidogrel-associated TTP do not appear to benefit from plasma exchange.
TRUE
Patients with clopidogrel-associated TTP do not appear to benefit from plasma exchange.
In TPE, most adverse effects were classified as _____________- and did not prevent the successful completion of the procedure.
Mild or moderate
In patients receiving plasma as the colloid exchange fluid, most adverse events include
Fever, chills, or urticaria
Muscle cramps, paresthesias, and mild nausea can be attributed to
Hypocalcemic toxicity
Plasma-ionized calcium decreases as a result of the rapid infusion of calcium-free pharmaceutical albumin and, in part, to the use of calcium-chelating agents as anticoagulants in plasma exchange procedures.
Refers to the removal of a patient’s red cells in exchange for donor red cells.
Red cell exchange
Clinical disorder is caused by an abnormality (inherited or acquired) of the patient’s red blood cells
The process when red cells are removed for therapeutic purposes, but not replaced with donor red cells
Erythrocytapheresis
Used in situations characterized by an untoward elevation in circulating red cell volume or in iron-overload states.
Advantage of automated red cell exchange than manual BT in Sickle cell disease
Mitigates the accumulation of iron while maintaining a low level of hemoglobin S in patients receiving chronic treatment
The World Health Organization has suggested that exchange transfusion be considered for nonimmune (ie, not previously exposed) patients with Plasmodium falciparum malaria who have any of the following characteristics:
- Greater than 30% parasitemia in the absence of clinical complications
- Greater than 10% parasitemia in the presence of severe disease
- Greater than 10% parasitemia and failure to respond to optimal chemotherapy after 12 to 24 hours
- Greater than 10% parasitemia and poor prognostic factors (elderly, late-stage parasites [schizonts] in the blood)
Red cell exchange is indicated as first-line therapy in Sickle cell disease:
- Acute vaso-occlusive stroke
- Acute chest syndrome refractory to standard management*
- Prophylaxis (primary or secondary) for vaso-occlusive stroke*
*red cell exchange may not be superior to simple transfusion
Intraerythrocytic parasite that can be efficiently removed using automated red cell exchange.
Babesia microti
Red cell exchange is recommended for patients with severe manifestations, high parasite burdens (>10%) or who are at high risk.
Miscellaneous Uses of Red Cell Exchange
- To prevent Rh sensitization of an Rh-negative woman who received emergency transfusion with Rh-positive red blood cells
- The macrolide immunosuppressant tacrolimus (and sirolimus) is highly erythrocyte-bound, and overdoses are not responsive to plasma exchange but can be mitigated using red cell exchange.
- Refractory methemoglobinemia in patients with glucose-6-phosphate dehydrogenase deficiency or after ingestion of strong oxidants
ASFA has designated symptomatic hyperleukocytosis (white blood cell count >100,000/μL in acute myeloid leukemias , white cell count >400,000/μL in acute lymphoblastic leukemia with leukostasis as a category ______ indication for therapeutic leukocytapheresis.
Asymptomatic hyperleukocytosis is designated a category _____ indication
Category II
Category III i
ASFA lists symptomatic thrombocytosis in patients with myeloproliferative neoplasms as a category____indication for thrombocytapheresis
Category II
Refers to the selective removal of platelets from a patient for therapeutic purposes using a blood-processing (apheresis) device
Thrombocytapheresis
A treatment process in which a patient’s mononuclear white blood cells are manipulated outside of the body such that their reinfusion into the patient results in downregulation of cytotoxic T-cell activity.
Extracorporeal photochemotherapy (ECP)
Uses of Extracorporeal photochemotherapy (ECP)
Palliative treatment of skin manifestations of cutaneous T-cell lymphoma unresponsive to other therapy
Treatment of acute cardiac allograft rejection and chronic graft-versus-host disease unresponsive to standard treatments
