1
Q
What is immune aplastic anemia (AA)?
A
AA is due to cytotoxic T-lymphocyte-driven immune destruction of hematopoietic stem and progenitor cells (HSPCs)
AA is a diagnosis of exclusion; other causes of bone marrow failure must be ruled out.
2
Q
What chromosome abnormalities are associated with myelodysplastic syndrome (MDS) over AA?
A
Chromosome abnormalities favor an MDS diagnosis over AA
Certain karyotypic changes like del13q, del20q, and trisomy 8 can be seen in AA.
3
Q
What specialized tests should be considered for new patients presenting with marrow failure?
A
Chromosome fragility tests and telomere length testing
Germline genetic testing for inherited bone marrow failure syndromes (IBMFS)-related genes should also be performed when possible.
4
Q
What is the treatment of choice for severe AA (SAA) in children and young adults?
A
Allogeneic stem cell transplantation from a matched sibling donor (MSD)
This is the preferred treatment for patients under 40 years of age.
5
Q
What therapy should be initiated for older patients with SAA who do not have sibling donors?
A
Immunosuppressive therapy (IST) with horse antithymocyte globulin (hATG), cyclosporine (CSA), plus eltrombopag (EPAG)
This should be started as soon as the diagnostic workup is completed.
6
Q
What has improved in recent years for patients with SAA regarding alternative donors?
A
Outcomes with matched unrelated donor (MUD) transplantation have improved
MUD HCT may be considered for treatment-naïve patients and is preferred for those who fail initial immunosuppression.
7
Q
What is a potential salvage option for patients with SAA who fail initial immunosuppression?
A
Haploidentical HCT may be considered appropriate
This remains investigational as up-front therapy.
8
Q
What percentage of patients with SAA who respond to IST experience relapses?
A
About 1/3 of patients
These relapses often respond well to reinstituted oral therapy with CSA +/- EPAG.
9
Q
What is the risk of clonal evolution to MDS in patients with SAA post-IST?
A
10% to 20% of patients may experience clonal evolution to MDS
Those with monosomy 7 have poor outcomes and should undergo HCT if possible.
10
Q
What prophylaxis should be used in patients with very severe AA (VSAA)?
A
Antifungal prophylaxis
The role of antibiotics, antivirals, and pneumocystis jirovecii pneumonia (PJP) prophylaxis is less well defined.
11
Q
What characterizes pure red cell aplasia (PRCA)?
A
Severe normochromic, normocytic, or macrocytic anemia with reticulocytopenia
This is defined by an absolute reticulocyte count of <10,000/μL.
12
Q
What are the two types of PRCA?
A
Congenital and acquired
Congenital includes Diamond-Blackfan Anemia (DBA); acquired is most commonly immune mediated.
13
Q
What occurs in transient erythroblastopenia of childhood?
A
Typically resolves over several months in otherwise healthy infants and young children
Treatment is supportive.
14
Q
What virus causes suppression of erythropoiesis and manifests anemia in immunosuppressed patients?
A
Parvovirus B19
Anemia manifests in immunosuppressed patients or those with shortened red cell survival.
15
Q
How is immune-mediated PRCA typically treated?
A
With immunosuppressive agents, typically cyclosporine with or without prednisone
16
Q
What do the bone marrow failure (BMF) syndromes comprise?
A
A group of clinically and pathologically distinct disorders associated with cytopenias due to failure of normal hematopoiesis
BMF presents as cytopenias and evidence of bone marrow hypoproliferation, including hypocellularity and reticulocytopenia.
17
Q
What is the clinical presentation of BMF?
A
Cytopenias and evidence of bone marrow hypoproliferation
This includes hypocellularity and reticulocytopenia.
18
Q
What are the common etiologies of BMF?
A
- Immune mediated
- Congenital due to germline genetic effects (IBMFS)
- Direct toxicity to the bone marrow
IBMFS refers to inherited bone marrow failure syndromes.
19
Q
What is aplastic anemia (AA)?
A
A hematopoietic stem and progenitor cell disorder with reduced bone marrow cellularity and decreased hematopoiesis
AA is typically associated with trilineage cytopenia with reticulocytopenia.
20
Q
How is severe aplastic anemia (SAA) defined?
A
Using the modified Camitta criteria by depression of blood counts involving at least 2 hematopoietic lineages
Criteria include absolute reticulocyte count <60 × 10^9/L, absolute neutrophil count <0.5 × 10^9/L, or platelet count <20 × 10^9/L, and bone marrow hypocellularity <30%.
21
Q
What distinguishes moderate AA from severe AA?
A
Moderate AA is characterized by depression of blood counts not fulfilling the definition of severe disease
Severe AA requires specific blood count criteria as outlined in the modified Camitta criteria.
22
Q
What is the main cause of aplastic anemia?
A
Most commonly immune mediated
AA may also be due to an inherited bone marrow failure syndrome (IBMFS).
23
Q
What is the age distribution of aplastic anemia incidence?
A
Bimodal age of incidence occurring most commonly in children and young adults, and again in adults over 60 years of age
AA is rare in Western Europe and the U.S. but more common in Asia.
24
Q
What is the incidence of aplastic anemia in Western Europe and the United States?
A
Fewer than 2 cases per million in the population per year
In Asia, the incidence is approximately 4 to 6 cases per million.
25
What factors are suspected to contribute to the higher incidence of AA in Asia?
Genetic disposition, specifically Asian human leukocyte antigen (HLA) type and nucleotide polymorphisms
## Footnote
No environmental factors have been identified despite focused epidemiology studies.
26
How does drug exposure relate to aplastic anemia?
The level of evidence linking AA to specific drugs is skimpy, and most cases are not found to be drug or toxin induced
## Footnote
Some drugs associated with AA include indomethacin, diclofenac, propylthiouracil, and chloramphenicol.
27
What percentage of AA cases is associated with hepatitis in Europe?
2% to 5% of cases
## Footnote
In East Asia, this figure is between 4% to 10%.
28
What is the nature of the hepatitis associated with aplastic anemia?
Seronegative hepatitis not caused by currently known hepatitis viruses
## Footnote
An immune pathogenesis following a putative trigger is suspected.
29
How does post-hepatitis AA evolve in relation to hepatitis episodes?
Typically evolves with a delay of several weeks to months after the episode of hepatitis
## Footnote
This is usually after the transaminitis has peaked and begins to trend down.
30
What is the main pathogenesis mechanism in immune AA?
Immune-mediated attack on HSPCs leading to deficiency in HSPCs
## Footnote
This results in low marrow CD34 cells and poor hematopoietic colony formation.
31
What cytokines are produced by activated cytotoxic T cells in immune AA?
TNFa and IFNg
## Footnote
These cytokines induce apoptosis via Fas and Fas ligand.
32
What is the inciting trigger for T-cell activation in immune AA?
Unknown
## Footnote
The exact cause of T-cell activation remains unidentified.
33
What is a surrogate measure of hematopoietic stem cells?
Long-term culture-initiating cells
## Footnote
This is reflected in diminished numbers of these cells in the marrow.
34
Which cells are frequently present in AA due to immune escape?
Granulocytes deficient in glycosyl phosphatidylinositol-anchored proteins (PNH cells)
## Footnote
These cells arise due to PIGA mutations.
35
What are typical symptoms of anemia in patients with AA?
Fatigue, weakness, pallor, headaches
## Footnote
These symptoms are primarily due to anemia.
36
What symptoms may arise from thrombocytopenia in AA?
Petechiae, epistaxis, gum bleeding
## Footnote
These symptoms are related to low platelet counts.
37
What may cause fever and serious infections in AA patients?
Neutropenia
## Footnote
Particularly in patients with neutrophil counts <0.2 × 10^9/L.
38
What is the typical patient profile for AA?
Previously healthy individual with no history of malignancy or cytotoxic drug exposure
## Footnote
AA often arises in individuals without prior health issues.
39
What findings on physical examination are atypical for AA?
Splenomegaly and hepatomegaly
## Footnote
Their presence should prompt consideration of alternative diagnoses.
40
What should be considered if a patient with AA has a family history of cytopenias?
Inborn Bone Marrow Failure Syndromes (IBMFS)
## Footnote
Family history may indicate genetic predispositions.
41
What is the criteria for diagnosing AA based on bone marrow cellularity?
Bone marrow with <25% normal cellularity or <50% normal cellularity with <30% hematopoietic cells
## Footnote
This is critical for the diagnosis of AA.
42
What is a common diagnostic test for suspected IBMFS in AA patients?
Telomere length and chromosome breakage studies
## Footnote
Particularly recommended for patients under 40 years of age.
43
How is chromosome breakage assessed for Fanconi Anemia (FA)?
By exposing cultured cells to diepoxybutane
## Footnote
Increased breakage indicates a diagnosis of FA.
44
What is the significance of detecting PNH clones in AA patients?
Helpful diagnostically, as PNH clones are rare in IBMFS
## Footnote
However, they can also be seen in MDS.
45
What is T-LGL and how is it characterized?
Clonal expansion of cytotoxic T-lymphocytes
## Footnote
It can coexist with immune AA or MDS.
46
What clinical clues suggest the presence of IBMFS in AA?
Younger age, multiorgan involvement, family history of hematologic disease
## Footnote
These factors can indicate a genetic syndrome.
47
What is the importance of germline genetic testing in AA?
Key component for diagnosing IBMFS
## Footnote
Omitting genetic testing can lead to missed diagnoses.
48
What are the most commonly seen somatic mutations in AA?
PIGA, BCOR, BCORL1, DNMT3A, ASXL1
## Footnote
Detection of these mutations can guide diagnosis toward immune AA.
49
True or False: Detection of a somatic variant in AA is diagnostic of MDS.
False
## Footnote
A somatic variant alone does not meet diagnostic criteria for MDS.
50
What is the common finding in peripheral blood of AA patients?
Pancytopenia with relative lymphocytosis
## Footnote
The blood is usually otherwise unremarkable.
51
What type of mutations are common in patients with suspected IBMFS?
EIF6 mutations in SDS, TERTp mutations in TBD
## Footnote
These mutations help in the diagnosis of specific syndromes.
52
What is the outcome for patients with SAA or very severe AA without treatment?
Almost all patients eventually die of infection or hemorrhagic complications.
53
What factors influence the decision to treat patients with AA?
Disease severity.
54
What are the definitive treatments for patients with SAA?
IST or allogeneic HCT.
55
Is therapy for non-severe AA established?
No, it is not established.
56
Under what condition is therapy for non-severe AA optional?
Except for cases with transfusion dependence.
57
Can patients with moderate AA spontaneously recover?
Yes, they can spontaneously recover normal hematopoiesis.
58
How soon can spontaneous remission of drug-induced AA occur?
Usually within 1 to 2 months of discontinuing the offending medication.
59
What influences the choice of treatment in SAA?
Patient age and availability of an appropriate donor for HCT.
60
What is the recommended treatment for younger patients (<40 years) with an MSD?
Allogeneic HCT.
61
What is the recommended treatment for older patients (>40 years) or those without an MSD?
IST.
62
What does standard IST for adults in the USA comprise?
Horse ATG and cyclosporine (CSA) with eltrombopag (EPAG).
63
Is the addition of EPAG considered standard of care in pediatric SAA?
No, it is not considered standard of care by many pediatric hematologists.
64
Why should therapy for SAA be administered without delay?
To prevent complications such as serious infection, bleeding, and HLA alloimmunization.
65
What should be done for almost all patients with newly diagnosed SAA?
Undergo HLA typing and identification of potential donors.
66
What is the purpose of supportive care in AA?
To sustain blood counts and prevent infectious complications.
67
What type of blood products should be used for transfusions in AA patients?
Irradiated, leukocyte-depleted blood products.
68
Why should transfusions from related family members be avoided?
Increased risk of subsequent graft rejection.
69
What is a major cause of death in patients with SAA?
Fungal and bacterial infections.
70
Should an active fungal infection delay definitive therapy?
No, it should not delay definitive therapy.
71
What is the role of preventive antibiotics in neutropenic patients?
Not well defined.
72
What patients are often maintained on antifungal prophylaxis?
VSAA patients with severe neutropenia (ANC <0.2).
73
What is the response of AA patients to recombinant erythropoietin?
Most patients do not respond due to elevated serum erythropoietin levels.
74
What is the role of granulocyte colony-stimulating factor (G-CSF) in AA?
Limited role; may improve neutrophil counts in some patients.
75
What was the outcome of adding G-CSF to standard ATG and cyclosporine therapy?
Did not improve rates of hematologic response rate or survival.
76
Are corticosteroids effective in treating AA?
No, they are ineffective and increase the risk of infection.
77
What is the limited role of corticosteroids in SAA?
Serum sickness prophylaxis with concurrent ATG administration.
78
What role do androgens have in the treatment of AA?
May have a supportive role but should not be used as primary upfront therapy.
79
What is the recommended action for adolescents and young adults with severe disease and an HLA-MSD?
They should proceed directly to hematopoietic stem cell transplantation (HCT) as this is usually curative.
80
What are the advantages of HCT over standard immunosuppressive therapy (IST)?
HCT offers a marked reduction in the risk of relapse and abrogation of the risk for the development of clonal disorders such as MDS and PNH.
81
What are the risks associated with hematopoietic stem cell transplantation?
The risks include acute and chronic graft-versus-host disease (GVHD).
82
What is the purpose of the pre-transplantation conditioning regimen in aplastic anemia (AA)?
To provide immunosuppression enabling donor stem cell engraftment and eliminate activated immune cells causing marrow aplasia.
83
What are common conditioning regimens for HCT?
* Cyclophosphamide × 4 days with ATG
* Cyclophosphamide × 2 days with fludarabine and ATG
84
What is the preferred source for the stem cell graft in AA?
Bone marrow is preferred due to increased risk of GVHD with peripheral blood stem cells.
85
Why is GVHD to be avoided in the AA setting?
Its occurrence is associated with decreased survival and long-term quality of life.
86
How do outcomes with matched-unrelated donor (MUD) HCT compare to sibling donors in children?
MUD HCT outcomes have compared favorably with those observed with sibling donors.
87
What is the risk of GVHD in MUD HCT compared to MSD HCT?
GVHD risk is twice as high in MUD versus MSD HCT.
88
What is the optimal time frame for performing MUD HCT?
MUD HCT must be performed in a timely manner, ideally within 2-3 months from diagnosis.
89
What are the important factors influencing outcomes after HCT?
* Patient age
* Time to transplant (optimal is <3 months from diagnosis)
* Type of allograft (HLA-matched sibling, unrelated, or mismatched donors)
90
What is the survival rate for children and adolescents undergoing HLA-matched sibling HCT?
Survival is excellent at >80-90%.
91
What is the long-term survival rate post HCT for adults over 40 years of age?
Long-term survival remains inferior at ~50%.
92
What has been observed about haploidentical HCT in recent studies?
It has had increasing success with lower toxicity than previously reported.
93
What dose of total body irradiation (TBI) is required to avoid graft failure in haploidentical HCT?
Moderately high dose TBI (400 cGy) is required.
94
What issues are associated with umbilical cord donors?
* Delayed engraftment
* Higher rates of graft rejection
95
What is the ongoing research focus related to umbilical cord donors?
Using cord expansion techniques to overcome obstacles.
96
True or False: The use of haploidentical HCT for treatment-naive SAA patients is currently standard practice.
False; it remains investigational.
97
What is the principal immunosuppressive agent used in SAA?
ATG (anti-thymocyte globulin)
## Footnote
ATG is produced by immunizing animals (horse or rabbit) with human T cells, leading to the production of antibodies against T cell antigens.
98
How is ATG manufactured?
By delivering human T cells to a horse or rabbit, which produces antibodies that are harvested and purified.
## Footnote
This results in a polyclonal animal serum with lymphocytotoxic properties.
99
What are the differences between rabbit ATG (rATG) and horse ATG (hATG)?
rATG has a longer half-life and results in more durable lymphocyte depletion compared to hATG.
## Footnote
Differences also include T-cell binding affinity, cytokine release, and T-cell subset depletion.
100
What was the initial treatment regimen for AA before the addition of EPAG?
hATG or CSA alone followed by hATG and CSA in combination.
## Footnote
This combination became the standard regimen until 2018.
101
What is the overall response rate at 3 months for patients receiving hATG/CSA?
Between 60% and 80%.
## Footnote
Response is defined as no longer meeting the Camitta criteria for SAA.
102
What defines a complete response in SAA treatment?
Significant hematologic recovery: Hb >10g/dL, ANC >1 × 10^9/L, platelets >100 × 10^9/L.
## Footnote
This indicates a successful treatment outcome.
103
How responsive is hepatitis-associated AA to immunosuppressive therapy?
Equally responsive as other forms of AA.
## Footnote
Most patients respond by 6 months, but some may take longer.
104
What were the results of adding further immunosuppression to h-ATG/CSA?
Disappointing; agents like mycophenolate mofetil, G-CSF, or sirolimus did not improve outcomes.
## Footnote
More lymphocytotoxic agents like rATG or alemtuzumab led to worse outcomes.
105
What is the current standard of care for SAA?
hATG/CSA and eltrombopag (EPAG).
## Footnote
EPAG is a thrombopoietin agonist with a response rate of ~40% in refractory SAA.
106
What was the overall response rate in the NIH trial for hATG/CSA/EPAG?
87% overall response rate and 39% complete response.
## Footnote
This was higher compared to 80% overall and 30% complete response for hATG/CSA alone.
107
What was the only severe adverse event related to EPAG in the NIH trial?
Rash in 2 patients.
## Footnote
The addition of EPAG was generally well tolerated.
108
What were the findings of the RACE study regarding hATG/CSA/EPAG?
Superior response rates were replicated in a European randomized control trial.
## Footnote
This supports the effectiveness of the combination treatment.
109
What are the response rates for oral therapy with combined CSA and EPAG alone?
46% as reported in the SOAR trial.
## Footnote
This is significantly less than when combined with ATG.
110
What were the response rates for androgens in immune AA treatment?
6% at 6 months and 29% at 20 months.
## Footnote
Overall survival at 5 years was 63%.
111
Are androgens recommended for the treatment of SAA?
No, unless no other suitable therapy is available.
## Footnote
They were historically used prior to ATG-based regimens.
112
What is the ongoing risk of relapse after IST with the addition of EPAG?
38% with EPAG compared to 33% without EPAG
113
What is the initial concern regarding EPAG in the context of clonal evolution?
Increased risk of clonal evolution
114
For how long should cyclosporine be continued after IST?
At least 6 months
115
Has the benefit of tapering or low-dose maintenance of CSA beyond 6 months been confirmed?
No, it has not been confirmed in prospective studies
116
What percentage of patients may respond to increased doses or reintroduction of CSA or EPAG?
About 60% to 70%
117
What percentage of patients remain chronically dependent on CSA or EPAG?
Approximately 25%
118
What is the preferred therapy for relapsed or refractory SAA patients?
HCT (Hematopoietic Cell Transplantation)
119
What is the response rate of administration of rATG and CSA in refractory and relapsed patients?
Approximately 30% in refractory and 60% to 65% in relapsed patients
120
What alternative IST has been studied in the relapsed/refractory setting?
Alemtuzumab
121
What is the trilineage response rate of Romiplostim in refractory AA?
39%
122
What is the most feared long-term complication after IST?
Clonal evolution to secondary hematological malignancies
123
What percentage of patients may evolve to MDS within the first 20 years after diagnosis?
Up to 10% to 20%
124
What is the most common cytogenetic abnormality associated with the development of MDS?
Monosomy 7
125
What is the overall survival rate at 5 years for patients who undergo HCT after developing MDS?
~60%
126
What are 'low-risk' clonal evolution events?
Cytogenetic abnormalities that do not signify progression to MDS
127
What are the most common cytogenetic abnormalities seen in low-risk clonal evolution?
* Deletion 13q
* Trisomy 8
128
What percentage of patients with AA have PNH clones at presentation?
Approximately 40% to 50%
129
What should all AA patients undergo yearly assessment for?
Development of PNH
130
What can trigger relapse in patients with known AA during pregnancy?
Pregnancy itself
131
What is the spontaneous remission rate in patients often upon birth or termination of pregnancy?
25% to 30%
132
What is the current overall survival rate for patients with SAA?
>90%
133
What can cause early mortality in SAA patients?
* Transplant related complications
* Severe cytopenia
134
What did a recent CIBMTR analysis reveal about SAA patients who survive at least 1 year post HCT or IST?
They have comparable longevity to the general US population
135
What is pure red cell aplasia (PRCA)?
Characterized by severe normochromic, normocytic, or macrocytic anemia with reticulocytopenia and either an absence of hemoglobin-containing cells (<3% of the nucleated marrow cells) or maturation arrest at the proerythroblast stage.
136
How is acquired PRCA classified?
As primary or secondary, depending on the absence or presence of an associated disease, infection, or drug.
137
What is the primary cause of most cases of acquired PRCA?
An aberrant immune response leading to suppression of red blood cell (RBC) development.
138
What is transient erythroblastopenia of childhood?
An acquired PRCA observed in infants and young children, usually between 6 and 36 months of age.
139
What are the common symptoms of transient erythroblastopenia of childhood?
Insidious onset of pallor or incidental finding of normocytic anemia.
140
What is the differential diagnosis for transient erythroblastopenia of childhood?
* Diamond-Blackfan anemia
* Parvovirus B19 infection
141
What is the typical resolution time for transient erythroblastopenia of childhood?
Resolves spontaneously within weeks or months with no sequelae.
142
What virus is associated with parvovirus B19 infection?
Causes erythema infectiosum (fifth disease).
143
What percentage of adults >50 years old have neutralizing antibodies against parvovirus B19?
More than 75%.
144
What does parvovirus B19 bind to in erythroid progenitors?
Blood group P antigen.
145
What is an aplastic crisis?
Clinically significant anemia that develops in immunosuppressed patients due to parvovirus B19 infection.
146
What is a common complication in patients receiving ABO-mismatched bone marrow transplants?
Approximately 20% develop a prolonged RBC aplasia due to recipient isohemagglutinins.
147
What treatment is effective for severe or life-threatening anemia in PRCA patients?
Plasma exchange using donor-type plasma and high doses of recombinant human ESAs.
148
What can cause PRCA related to drug use?
Development of anti-EPO antibodies during treatment with recombinant human ESAs.
149
What is the incidence of PRCA in patients with thymoma?
Approximately 5%.
150
What is the relationship between thymoma and PRCA?
Thymoma occurs in approximately 10% to 15% of patients presenting with PRCA.
151
What is recommended for patients with idiopathic PRCA?
Lymphocyte immunophenotyping to assess for malignancy.
152
What are the symptoms of acquired PRCA?
Symptoms are related to the severity of anemia, with pallor being common. Physical examination is often normal in acquired primary PRCA.
153
What findings may be present in acquired secondary PRCA?
Findings may include hepatomegaly, splenomegaly, or lymphadenopathy.
154
What is the initial suggestion for diagnosing acquired PRCA?
Normochromic, normocytic, or macrocytic anemia with reticulocytopenia (absolute reticulocyte count of <10 000/μL).
155
What are the typical white blood cell and platelet counts in acquired PRCA?
Generally normal.
156
What is used to establish the diagnosis of PRCA?
Bone marrow biopsy and aspirate.
157
What may be observed in the marrow aspirate during parvovirus B19 infection?
Giant pronormoblasts.
158
What can the marrow show if PRCA is secondary to T-LGL or another lymphoproliferative disorder?
Lymphocytic infiltration.
159
What should be included as part of the initial workup for PRCA?
Routine karyotype and interphase fluorescence in situ hybridization panel to exclude an underlying MDS.
160
What additional studies may be considered in the diagnosis of PRCA?
CT scan of the chest, EPO level, and parvovirus B19 DNA testing by polymerase chain reaction.
161
What is the treatment for immunologically mediated PRCA?
Immunosuppressive therapy.
162
What percentage of patients achieve remission with immunosuppressive therapy for PRCA?
60% to 70%.
163
What is the variable response treatment for PRCA associated with thymoma?
Thymectomy; many patients also require immunosuppression.
164
Is there a benefit to removing a normal thymus in patients with PRCA without thymoma?
No benefit.
165
What is the first-line treatment for idiopathic cases of PRCA?
Cyclosporine +/- prednisone.
166
What are other potential therapies for PRCA?
* Oral cyclophosphamide +/- prednisone
* Azathioprine
* Sirolimus
167
What therapies may be considered when T-LGL is present?
T-cell directed therapies such as horse antithymocyte globulin and alemtuzumab.
168
What therapy may be considered for B-cell lymphoproliferative disorder?
Anti-CD20 therapy (e.g., rituximab).
169
What is a reasonable duration to assess response to immunosuppressive therapy?
3 to 6 months.
170
What may be done for responsive patients after treatment?
Treatment may continue for 3 to 6 months before tapering immunosuppression.
171
What may be utilized in rare refractory cases of PRCA?
Hematopoietic cell transplantation (HCT).
172
How can PRCA caused by parvovirus B19 in immunosuppressed individuals be treated?
Intravenous immunoglobulin (IVIG).
173
Are antiviral agents effective for treating PRCA caused by parvovirus B19?
No data to suggest efficacy.
174
What is a common issue after withdrawal of therapy in PRCA patients?
Many patients relapse and require long-term immunosuppression.
175
What treatment can be restarted for patients who relapse?
Prior effective therapy.
176
How are patients with severe symptomatic anemia treated?
Transfusion therapy.
177
What risks are associated with transfusion therapy in PRCA?
* Iron overload
* Alloantibody formation
178
What are common causes of death in nonresponding PRCA patients?
* Infection
* Iron overload
* Cardiovascular events
ASH SAP 9 ED
flashcards
Decks in class (17)
# Cards
-
2 Outpatient Hematology104
-
15 Intro VTE228
-
23 ITP151
-
27 Laboratory Hematology243
-
30 Basics of SCT104
-
31 Auto Transplant149
-
32 Allo Transplant151
-
33 Adoptive Cellular Therapy83
-
37 MPNs190
-
39 Acquired BMF178
-
40 MDS and CHIP252
-
41 AML129
-
42 ALL104
-
43 Hodkgin Lymphoma131
-
47 CLL224
-
48 MGUS and Myeloma346
-
49 AL Amyloid, Waldenstrom, POEMS152
