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WILLIAM'S HEMATOLOGY > 30 Therapeutic Apheresis: Indications, Efficacy and Complications > Flashcards

30 Therapeutic Apheresis: Indications, Efficacy and Complications Flashcards

1
Q

Category I

A

Apheresis is an accepted first-line therapy for these disorders.

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2
Q

Category II

A

Apheresis is an accepted second-line therapy for these disorders.

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3
Q

Category III

A

Individualize decision making. The optimal role of apheresis has not been conclusively determined in these disorders.

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4
Q

Category IV

A

Published evidence indicates that apheresis is ineffective or harmful in these disorders. Institutional review board approval is desirable if apheresis is planned.

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5
Q

The term that refers to the removal of plasma from the circulation by manual or automated methods

A

Plasmapheresis

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6
Q

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

A

Plasma exchange

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7
Q

The “sweet spot” for plasma exchange procedures is the processing of between ___________ plasma volumes.

A

1.0 and 1.5

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8
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

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8
Q

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.

A

Waldenström macroglobulinemia

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9
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

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10
Q

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.

A
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10
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

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10
Q

Waldenström macroglobulinemia: - Symptoms typically emerge when serum viscosity rises above ________ relative viscosity units (normal being 1.4-1.8).

A

4.0 relative viscosity units

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11
Q

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.

A

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.

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12
Q

Are immunoglobulins or complexes of immunoglobulins that reversibly precipitate when exposed to temperatures below 37°C.

A

Cryoglobulins

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13
Q

Associations of Types of Cryoglobulinemia

A

Type I: lymphoproliferative disorders

Type II: hepatitis C

Type III: chronic infections or autoimmune disorders

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14
Q

Types of Cryoglobulinemia

A

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

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15
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

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15
Q

Types of Cryoglobulinemia

A

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

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15
Q

TRUE OR 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
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16
Q

Results from combination of free light chains with Tamm-Horsfall mucoprotein in the distal nephron and the resultant precipitation of obstructing casts

A

Myeloma cast nephropathy (“myeloma kidney”)

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17
Q

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.

A

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.

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18
Q

A medical emergency that presents with microangiopathic hemolytic anemia and thrombocytopenia

A

Idiopathic thrombotic thrombocytopenic purpura (TTP)

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19
Q

In acquired idiopathic TTP, this enzymatic defect is caused by an autoantibody inhibitor of __________that results in severe deficiency of the enzyme

A

ADAMTS-13

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19
Q

TPE, using ________ as the colloid exchange fluid, was the only therapy for TTP that has been demonstrated highly effective in a randomized clinical trial.

A

Human plasma

20
Q

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.

A

Rituximab

21
Q

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.

A

Caplacizumab

22
Q

A thrombotic microangiopathy with acute oliguric or anuric renal failure, is rarely associated with severe deficiency of ADAMTS-13.

A

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

23
Q

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 nd is more appropriately treated with_________________.

A

Eculizumab

23
Q

The two most common drugs reported to the FDA as associated with TTP are

A

Ticlopidine and clopidogrel

24
Q

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 nd is more appropriately treated with_________________.

A

Eculizumab

24
Q

Autoantibodies to ADAMTS-13 are seen in ticlopidine-associated TTP

A
24
Q

Types of Cryoglobulinemia

A

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

24
Q

In acquired idiopathic TTP, this enzymatic defect is caused by an autoantibody inhibitor of __________that results in severe deficiency of the enzym

A

ADAMTS-13

24
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

25
Q

Autoantibodies to ADAMTS-13 are seen in ____________-associated TTP

A

Ticlopidine-associated TTP

25
Q

TRUE OR FALSE

Patients with clopidogrel-associated TTP do not appear to benefit from plasma exchange.

A

TRUE

Patients with clopidogrel-associated TTP do not appear to benefit from plasma exchange.

26
Q

In TPE, most adverse effects were classified as _____________- and did not prevent the successful completion of the procedure.

A

Mild or moderate

27
Q

In patients receiving plasma as the colloid exchange fluid, most adverse events include

A

Fever, chills, or urticaria

28
Q

Muscle cramps, paresthesias, and mild nausea can be attributed to

A

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.

29
Q

The process when red cells are removed for therapeutic purposes, but not replaced with donor red cells

A

Erythrocytapheresis

29
Q

Refers to the removal of a patient’s red cells in exchange for donor red cells.

A

Red cell exchange

Clinical disorder is caused by an abnormality (inherited or acquired) of the patient’s red blood cells

30
Q

The process when red cells are removed for therapeutic purposes, but not replaced with donor red cells

A

Erythrocytapheresis

Used in situations characterized by an untoward elevation in circulating red cell volume or in iron-overload states.

31
Q

Advantage of automated red cell exchange than manual in Sickle cell disease

A

Mitigates the accumulation of iron while maintaining a low level of hemoglobin S in patients receiving chronic treatment

32
Q

Red cell exchange is indicated as first-line therapy in

A
33
Q

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:

A

○ 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, or

○ greater than 10% parasitemia and poor prognostic factors (elderly, late-stage parasites [schizonts] in the blood).

33
Q

Red cell exchange is indicated as first-line therapy in Sickle cell disease:

A

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

34
Q

Intraerythrocytic parasite can be efficiently removed using automated red cell exchange.

A

Babesia microti

Red cell exchange is recommended for patients with severe manifestations, high parasite burdens (>10%) or who are at high risk.

34
Q

Miscellaneous Uses of Red Cell Exchange

A

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

35
Q

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.

A

Category II

36
Q

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

A

Category II

Category III i

37
Q

In patients receiving plasma as the colloid exchange fluid, most adverse events include

A

Fever, chills, or urticaria

37
Q

Types of Cryoglobulinemia

A

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

37
Q

Red cell exchange is indicated as first-line therapy in Sickle cell disease:

A

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

37
Q

In acquired idiopathic TTP, this enzymatic defect is caused by an autoantibody inhibitor of __________that results in severe deficiency of the enzym

A

ADAMTS-13

37
Q

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 nd is more appropriately treated with_________________.

A

Eculizumab

37
Q

Class I Indications for TPE

A

Catastrophic antiphospholipid syndrome
Cutaneous T-cell lymphoma; mycosis fungoides; Sézary syndrome: Erythrodermic
Erythrocytosis: Primary (Polycythemia vera)
Hereditary hemochromatosis
Hyperviscosity in monoclonal gammopathies: Symptomatic; Prophylaxis for rituximab
Sickle cell disease: Acute stroke amnd Stroke prophylaxis
Thrombotic microangiopathy: Drug-associated: Ticlopidine
TTP
Complement mediated; Factor H autoantibody

38
Q

ASFA lists symptomatic thrombocytosis in patients with myeloproliferative neoplasms as a category____indication for thrombocytapheresis

A

Category II

38
Q

Refers to the selective removal of platelets from a patient for therapeutic purposes using a blood-processing (apheresis) device

A

Thrombocytapheresis

39
Q

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.

A

Extracorporeal photochemotherapy (ECP)

40
Q

Uses of Extracorporeal photochemotherapy (ECP)

A

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

WILLIAM'S HEMATOLOGY (89 decks)

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