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WILLIAM'S HEMATOLOGY > 80 Hematologic Manifestations of Human Immunodeficiency Virus and the Acquired Immunodeficiency Syndrome > Flashcards

80 Hematologic Manifestations of Human Immunodeficiency Virus and the Acquired Immunodeficiency Syndrome Flashcards

1
Q

HIV-1, the virus that causes AIDS, is a ___________ that originated as a simian immunodeficiency virus (SIV) in chimpanzees and entered the human population in the early 20th century in equatorial Africa

A

Lentivirus

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2
Q

HIV-1 actually comprises four distinct viruses

A

Types M, N, O, and P

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3
Q

The viral type responsible for the HIV-1 pandemic, was detected in a tissue sample from 1959 and probably entered the human population in or around Kinshasa, Democratic Republic of Congo (then Leopoldville, Belgium Congo) between 1910 and 1930

A

Group M

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4
Q

The result of cross-species transmission of SIV from sooty mangabeys to humans

A

HIV-2

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5
Q

HIV1 or HIV2

Progress with disease more slowly and have lower plasma viral loads (often nondetectable)

A

HIV2

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6
Q

The globally predominant viral strain and is further divided into nine subtypes and many more recombinant viruses (circulating recombinant forms [CRFs]) with some geographic localization

A

Group M

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7
Q

Geographic localization of Group M

Subtypes A and D:
Subtype C:
Subtype B:
CRF01:

A

Geographic localization of Group M

Subtypes A and D: East Africa
Subtype C: most prevalent subtype globally, in South Africa, India, and Asia
Subtype B: Caribbean, the Americas, and Western Europe
CRF01:Southeast Asia

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8
Q

Predominant mode of transmission in the following areas:

United States, Northern Europe, Australia, and parts of Central and South America:
Sub-Saharan Africa:
Southern and Eastern Europe and Southeast Asia:

A

Predominant mode of transmission in the following areas:

United States, Northern Europe, Australia, and parts of Central and South America: sexual contact between men

Sub-Saharan Africa: heterosexual spread

Southern and Eastern Europe and Southeast Asia:injection drug use followed by sexual transmission

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9
Q

Eighty percent of HIV infections occur via ____________________when cell-free and cell-associated virions transverse the epithelium to gain access to macrophages, Langerhans cells, dendritic cells, and CD4-expressing T lymphocytes.

A

Mucosal transmission during sex

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10
Q

To infect most cells, HIV must bind to CD4 and one of two major coreceptors:

A

CCR5 or CXCR4

** In most cases, CCR5-utilizing viral strains are those that are transmitted and predominate early in disease

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11
Q

Replication of HIV occurs in

A

tissue macrophages and dendritic cells

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12
Q

Typically, the asymptomatic phase of chronic infection will last for ________, although there is great interindividual variation dictated by the effectiveness of the immune response in controlling HIV replication

A

8 to 10 years

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13
Q

Those who maintain CD4+ T-cell counts >500 for 5 years without therapy

A

Long-term nonprogressors

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14
Q

Those with low or nondetectable plasma HIV RNA without treatment

A

Elite controllers

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15
Q

Tumors classified as AIDS-defining malignancies

A

Kaposi sarcoma
Cervical cancer
Certain subtypes of non-Hodgkin lymphoma (NHL) (initially Burkitt lymphoma)
Immunoblastic lymphoma
Primary CNS lymphoma

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16
Q

HIV STAGING: Infection within the previous 6 months

A

Stage 0

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17
Q

HIV STAGING: CD4 count ≥500 cells/μL (or ≥26%)

A

Stage 1

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18
Q

HIV STAGING: CD4 count 200–499 cells/μL
(or 14%–25%)

A

Stage 2

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19
Q

HIV STAGING: AIDS-defining condition or CD4 count <200 cells/μL (or <14%)

A

Stage 3

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20
Q

Opportunistic Infections by CD4 Count

> 500 cells/μL

A

Any condition that can occur in HIVuninfected
persons, eg, bacterial pneumonia, tuberculosis, varicella zoster, herpes simplex virus

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21
Q

Opportunistic Infections by CD4 Count

350–499 cells/μL

A

Thrush, seborrheic dermatitis, oral hairy
leukoplakia, molluscum contagiosum

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22
Q

Opportunistic Infections by CD4 Count

200–349 cells/μL

A

Kaposi sarcoma, lymphoma

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23
Q

Opportunistic Infections by CD4 Count

100–199 cells/μL

A

Pneumocystis pneumonia, Candida esophagitis, cryptococcal meningitis

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24
Q

Opportunistic Infections by CD4 Count

<100 cells/μL

A

Toxoplasma encephalitis, disseminated, Mycobacterium avium complex, progressive
multifocal leukoencephalopathy, cytomegalovirus retinitis, primary CNS lymphoma, microsporidia

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25
Q

TRUE OR FALSE

All HIV-infected persons be offered ART regardless of CD4 count

A

TRUE

All HIV-infected persons be offered ART regardless of CD4 count

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26
Q

Primary Prophylaxis: Pneumocystis pneumonia

CD4 <200 cells/μL or <14% or oral candidiasis or an AIDS-defining illness

A

Trimethoprimsulfamethoxazole or dapsone or
aerosolized pentamidine

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27
Q

Primary Prophylaxis: Tuberculosis

Purified protein derivative >5 mm or positive
interferon-γ release assay

A

Isoniazid and pyridoxine

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28
Q

Primary Prophylaxis: Toxoplasmosis

Immunoglobulin G+ and CD4 <100 cells/μL

A

Trimethoprimsulfamethoxazole or dapsone plus pyrimethamine plus leucovorin

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29
Q

Primary Prophylaxis: Mycobacterium avium complex

CD4 <50 cells/μL

A

Azithromycin or Clarithromycin

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30
Q

An acute inflammatory reaction as a result of reconstitution of the immune system in the presence of organisms or foreign antigens

A

Immune reconstitution inflammatory syndrome (IRIS)

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31
Q

Risk factors for the development of IRIS

A

Low baseline CD4 count
More severe disease
A short interval between treatment of the opportunistic infection and initiation of ART

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32
Q

Treatment of IRIS

A

Treatment of the underlying infection or condition
Continued ART
Antiinflammatory medication, such as glucocorticoids

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33
Q

The impediment to cure for nearly all HIV-infected people

A

The persistence of replication-competent but transcriptionally silent HIV proviral DNA in long-lived resting cells (the HIV latent reservoir)

34
Q

TRUE OR FALSE

All patients who present to the hematologist with NHL, Hodgkin lymphoma, idiopathic thrombocytopenic purpura (ITP), or other malignancies should be tested for HIV

A

TRUE

All patients who present to the hematologist with NHL, Hodgkin lymphoma, idiopathic thrombocytopenic purpura (ITP), or other malignancies should be tested for HIV

**5% of those with diffuse large B-cell lymphoma (DLBCL) and 22% of patients with Burkitt lymphoma in the United States are HIV-positive

35
Q

The most common malignancy in people living with HIV worldwide.

A

Kaposi sarcoma

36
Q

AIDS-Defining Malignancies and Oncogenic Viruses

Kaposi sarcoma:
Aggressive non-Hodgkin lymphoma:
Primary CNS lymphoma:
Invasive cervical cancer:

A

AIDS-Defining Malignancies and Oncogenic Viruses

Kaposi sarcoma: HHV8
Aggressive non-Hodgkin lymphoma: EBV, HHV8
Primary CNS lymphoma: EBV
Invasive cervical cancer: HPV

37
Q

TRUE OR FALSE

HIV-associated Burkitt lymphoma is approximately one-third as common as HIV-associated DLBCL in the Western world, and occurs at a lower CD4 count.

A

FALSE

HIV-associated Burkitt lymphoma is approximately one-third as common as HIV-associated DLBCL in the Western world, and occurs at a HIGHER CD4 count.

38
Q

Burkitt lymphoma in HIV-negative people, and involves translocation of the _____________ with one of the immunoglobulin (Ig) genes on chromosomes 2, 14, or 22, resulting in overexpression of Myc

A

Myc gene on chromosome 8

39
Q

More than ______of patients with HIV-associated Burkitt lymphoma present with stage IV disease and extranodal sites are often involved.

A

More than 80%

40
Q

A common feature of CNS involvement of Burkitt lymphoma

A

Cranial nerve palsies

41
Q

TRUE OR FALSE

As in the HIV-negative setting, CHOP is not adequate treatment for Burkitt lymphoma and should not be used.

A

TRUE

As in the HIV-negative setting, CHOP is not adequate treatment for Burkitt lymphoma and should not be used.

42
Q

Primary CNS lymphoma in HIV-positive patients usually have a CD4 count of less than_______

A

CD4 count of less than 50 cells/μL and often of less than 20 cells/μL.

43
Q

The pathophysiology of HIV-associated primary CNS lymphoma is related to _______ virus, which is detectable in virtually all case

A

EBV

44
Q

The pathophysiology of HIV-associated primary CNS lymphoma is related to _______ virus, which is detectable in virtually all case

A

EBV

45
Q

In one series, the most common symptom of CNS lymphoma

A

Headache

46
Q

Characteristic features on magnetic resonance imaging of the brain in primary CNS lymphoma

A

A single to several mass lesions in the subcortical white matter

47
Q

Anatomic sites commonly involved are predominantly in primary CNS lymphoma

A

Cerebral cortex and periventricular area

Cerebellar or brainstem involvement is rare

48
Q

TRUE OR FALSE

Detection of EBV in the CSF supports, but does not confirm, the diagnosis of primary CNS lymphoma

A

TRUE

Detection of EBV in the CSF supports, but does not confirm, the diagnosis of primary CNS lymphoma

49
Q

Can help distinguish primary CNS lymphoma from other common causes of focal brain lesions in profoundly immunosuppressed patients with HIV, namely, cerebral toxoplasmosis and other infections.

A

PET-CT of the brain

50
Q

A rare and very aggressive B-cell NHL with plasmacytic differentiation that often involves the oral cavity, typically the gingiva and the palate.

A

Plasmablastic lymphoma

51
Q

An aggressive B-cell lymphoma characterized by lymphomatous effusions in body cavities, most commonly pleural effusion, followed by ascites and pericardial effusion or multiple body cavities; lymph nodes, marrow, and skin can also be involved.

A

Primary effusion lymphoma (PEL)

52
Q

A solid variant of PEL that presents without effusion, but with lymph node, gastrointestinal, skin, or liver involvement and shares epidemiologic and immunophenotypic characteristics with the classical PEL.

A

Extracavitary PEL

53
Q

100% of PEL are ___________–positive and approximately 80% are EBV-positive

A

human herpesvirus-8 (HHV8)

54
Q

HHV8 plays a key pathophysiologic role, possibly by elaboration of a viral homologue of _______ and a viral homologue of ____________

A

FLICE inhibitory protein
interleukin (IL)-6

55
Q

Other HHV8-related disorders

A

Castleman disease
Kaposi sarcoma
Aggressive NHL

56
Q

ART that causes marrow suppression and should be avoided in patients receiving myelosuppressive chemotherapy

A

Zidovudine

57
Q

A rare polyclonal lymphoproliferative disorder characterized by periodic flares (an inflammatory illness accompanied by lymphadenopathy and splenomegaly), and a high risk of progression to lymphoma

A

Castleman disease

58
Q

The pathophysiology of Castleman disease is related to

A

IL-6

59
Q

The morphology of multicentric Castleman disease in HIV-positive patients

A

Plasmablastic cells in the mantle zone of the follicles; HHV8 is detectable in the plasmablasts

60
Q

The backbone of treatment of Castleman disease

A

Rituximab

61
Q

An anti–IL-6 monoclonal antibody approved by the FDA for treatment of Castleman disease in HIV-negative patients but not in HIV-positive patients

A

Siltuximab

62
Q

An anti–IL-6 receptor monoclonal antibody FDA-approved for use in rheumatoid arthritis but not for treatment of Castleman disease

A

Tocilizumab

63
Q

Most common lymphoma in patients with HIV

A

DLBCL

64
Q

A syndrome characterized by an inflammatory illness similar to a flare of Castleman disease, but without the pathologic diagnosis of Castleman disease

A

KSHV-associated inflammatory cytokine syndrome (KICS)

65
Q

Immunohistochemical and Molecular Diagnostic Findings

Diffuse large-B-cell lymphoma

A

Immunohistochemical and Molecular Diagnostic Findings

Diffuse large-B-cell lymphoma: CD20+, may have c-myc translocation

66
Q

Immunohistochemical and Molecular Diagnostic Findings

Burkitt’s lymphoma

A

CD20+, CD10+, c-myc translocation

67
Q

Immunohistochemical and Molecular Diagnostic Findings

AIDS-related primary CNS lymphoma

A

CD20+, EBV+

68
Q

Immunohistochemical and Molecular Diagnostic Findings

Primary effusion lymphoma

A

DC20−, KSHV+, EBV+ (in approximately 80% of cases)

69
Q

Immunohistochemical and Molecular Diagnostic Findings

Plasmablastic lymphoma

A

CD20−, EBV+, may have c-myc translocation

70
Q

Immunohistochemical and Molecular Diagnostic Findings

KSHV-associated multicentric Castleman’s disease

A

KSHV+, lambda-restricted plasmablasts; a proportion of infected cells are viral IL-6+

71
Q

Immunohistochemical and Molecular Diagnostic Findings

Classic Hodgkin lymphoma

A

Often EBV+, Reed-Sternberg cells

72
Q

Results from failure to regulate the immune response, resulting in excess activation of T lymphocytes, increased cytokine secretion, and hyperactivation of macrophages

A

Hemophagocytic syndrome

73
Q

An ART that can cause macrocytic anemia and leukopenia and is not commonly used at present because of its toxicity.

A

Zidovudine

74
Q

Prophylactic drug used for P. jiroveci prophylaxis, can cause hemolytic anemia in patients with glucose-6-phosphate dehydrogenase deficiency

A

Dapsone or trimethoprim-sulfamethoxazole

75
Q

Drug used to treat herpes simplex virus, varicella zoster, CMV, and sometimes HHV8, often cause pancytopenia.

A

Ganciclovir or valganciclovir

76
Q

Drug for PCP prophylaxis that can also cause myelosuppression and pancytopenia

A

Trimethoprim-sulfamethoxazole

77
Q

The optimal treatment for HIV-associated anemia

A

Initiation of ART

78
Q

An FDA-approved indication for erythropoietin in HIV patients

A

Anemia caused by zidovudine and an erythropoietin level of 500 mU/mL or less

79
Q

The primary treatment of HIV-associated ITP

A

Initiation of ART

80
Q

Treatment option in HIV-associated ITP with severe thrombocytopenia, Rh+ and has an intact spleen

A

IV anti-D

81
Q

Major side effect of anti-D treatment

A

Drop in the hemoglobin (significant hemolysis)

82
Q

TRUE OR FALSE

In HIV-associated ITP, glucocorticoids are less attractive because of the potential to decrease CD4 counts, increase the risk of infection, and increase the risk of Kaposi sarcoma progression.

A

TRUE

In HIV-associated ITP, glucocorticoids are less attractive because of the potential to decrease CD4 counts, increase the risk of infection, and increase the risk of Kaposi sarcoma progression.

WILLIAM'S HEMATOLOGY (89 decks)

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