Nu 545- Unit 4 Study Guide
Infectious Mononucleosis p. 966
A benign, acute, self-limiting lymphoproliferative clinical syndrome characterized by acute viral
infection of B lymphocytes (B cells). Associated with several tumors, most commonly specific
lymphomas and nasopharyngeal carcinoma. Most common cause- EBV.
Major manifestations of EBV induced IM: Pharyngitis, lymphadenopathy, Fever.
Other viral causes for IM: CMV, adenovirus, HIV, Hep A, Influenza A&B, Rubella.
Transmission of EBV: Saliva (Kissing Disease), secretions of genitalia rectum resp tract & blood.
No aerosol transmission.
Disease begins with widespread infection of B lymphocytes which have receptors for EBV. Virus
initially infects oropharynx, nasopharynx, and salivary epithelial cells then spreads to lymphoid
tissue and B cells. Infection of B cells allows the virus to enter the bloodstream, then the virus
spreads systemically.
Patho of Infectious Mononucleosis p. 966
In the immunocompetent patient, unaffected B cells produce antibodies (IgG, IgM, IgA) against
the virus. There is a massive activation of proliferation of cytotoxic T cells (CD8) directed against
EBV infected cells. Immune response against EBV is largely responsible for cellular proliferation
in the lymphoid tissue (lymph nodes, spleen, tonsils, liver). Sore throat and fever are the earliest
manifestations d/t inflammation at the site of viral entry and initial infection, usually the mouth
and throat.
Complications of infection Mononucleosis p. 967 fig 30.1
Lymphadenitis
hepatitis
Splenitis
Pneumonitis
Meningitis
Encephalitis
Clinical manifestations of infectious mononucleosis p. 967
Incubation period: 30-50 days (4-8 weeks), then a 3-5 day prodrome of fever, malaise,
arthralgias (joint pain). These symptoms vary in severity for the next 7-20 days.
At time of dx the patient has a classic triad of symptoms: fever, pharyngitis, lymphadenopathy of
cervical lymph nodes.
Pharyngitis: whitish, greyish green thick exudate.
,Severe complications: meningitis, encephalitis, guillain barre syndrome, bell's palsy, optic
neuritis, mental impairment, transverse myelitis, cerebellar ataxia, demyelinating disease.
Ocular manifestations: eyelid/periorbital edema, dry eyes, keratitis, uveitis, conjunctivitis,
retinitis, oculoglandular syndrome, choroiditis, papillitis, ophthalmoplegia. In child: Reye
syndrome
Pulmonary involvement: RARE- hilar and mediastinal lymphadenopathy, interstitial pneumonitis,
pleural effusions, pneumonia and resp fail in immunocompromised patients.
Older patient with 2 weeks of temp that can't be explained EBV should be suspected
Eval and Tx of infectious mononucleosis p. 967
Children present w/: fever, sore throat, lymphadenitis,
Young adults present w/: malaise, fatigue, lymphadenopathy and fever of unknown origin.
Blood contains increased # of atypical lymphocytes.
Dx based on Hoagland's criteria: 50% lymphocytes, 10% atypical lymphocytes in the blood with
presence of fever, pharyngitis, adenopathy confirmed by a + serologic test.
Serological test: heterophile antibodies, Monospot test (limited bc CMV, adenovirus,
toxoplasmosis also produce heterophilic antibodies causing false +).
Tx: IM is usually self limiting and intervention is rarely required. Rest & alleviation of symptoms.
No ASA used with child or adolescent d/t reye syndrome.
Pharyngitis of Strep origin tx w/ PCN or erythromycin. NO ampicillin (causes rash in patients
with IM)
Avoid strenuous activities. Steroids only with severe complications. Acyclovir with
immunocompromised.
Leukemia p. 968
A clonal malignant disorder of leukocytes in the bone marrow and usually but not always of the
blood. Common feature is an uncontrolled proliferation of malignant leukocytes causing an
overcrowding of bone marrow and decreased production of functional normal hematopoietic
cells. Thus it has been termed as an accumulation disorder as well as proliferation disorder.
Acute Leukemia p. 968
Characterized by undifferentiated or immature cells, usually a blast cell, and the onset of
disease is abrupt and rapid with a short survival time.
Chronic Leukemia p. 968
The predominant cell is more differentiated but does not function normally, with a relatively slow
progression
4 types of leukemia p. 968
,Acute Lymphocytic (ALL)
Acute Myelogenous (ALM)
Chronic Lymphocytic (CLL)
Chronic Myelogenous (CML)
Acute Leukemia p. 970
ALL- an aggressive fast growing leukemia with too many lymphoblasts (immature WBC) found
in the blood and bone marrow.
ALM- Aggressive fast growing leukemia with an excessive number of myeloblasts (immature
WBC that are not lymphoblasts) found in bone marrow and blood.
Acute leukemia is a result of B and T cell origin
ALL of B cell origin occurs mainly in children involving the mutation required for B cell
development (ETV6 & RUNX1).
ALL of T cell origin- Mutation in NOTCH1
ALL- defined by > 30% of lymphoblasts in bone marrow or blood
ALM- Epigenome alteration; incidence peaks in 6th decade of life
Clinical Manifestations:
ALL- w/in days to a few weeks stormy onset:
S/S generally the same with all acute leukemias:
Table 30.2
Fatigue from bone marrow depression, bleeding from thrombocytopenia, fever from infection.
Infection is usually gram-negative bacilli: E Coli, Pseudomonas aeruginosa, Klebsiella
pneumoniae.
Liver, spleen and lymph node enlargement more common in ALL
CNS S/S: HA, V, papilledema, facial palsy, blurred vision, auditory disturbances, meningeal
irritation
Prophylaxis is key with CNS involvement: radiation/chemo but not needed with ALM bc only 5%
of ALM have CNS involvement
Eval/Tx:
dx made by exam of blood cells and bone marrow.
Blood smear will show low RBC/PLt count and presence of leukemic blast cells.
Bone marrow will show 60-100% blast cells
Chemotherapy is Tx of choice
Supportive measures: Blood transfusion, ATB, antifungals, antivirals.
Stem Cell transplant
ATRA- made in the body from Vit A and helps cells grow and differentiate. It is a
chemotherapeutic agent used for tx.
The 5 year survival rate for ALL has increased to 65% in adults and 85% in children since 1970.
The Philadelphia chromosome is a poor prognostic indicator.
Myelosuppression is a consequence of leukemia and tx. requires hematologic support with
blood products.
, Chronic Leukemia p. 973
CLL- Slow growing Ca in which too many immature lymphocytes are found mostly in the blood
and bone marrow.
Chronic leukemia has a longer life expectancy of several years after dx.
Cause of CML is exposure to ionizing radiation.
The Philadelphia chromosome is present in 95% of CML and presence of BCR-ABL1 protein is
responsible for initiation of CML.
CLL- malignant transformation and progressive accumulation of B lymphocytes
CLL cells that accumulate in the marrow do not interfere with normal blood cell production to the
extent found in acute leukemias
Clinical Manifestations:
Lymphadenopathy, suppression of humoral immunity and increased infection with encapsulated
bacteria, < neutrophils which > risk for infection, CNS involvement rare.
CML- 3 phases of disease: Chronic phase 2-5 years symptoms not apparent; accelerated phase
6-18 months primary symptoms develop (excessive proliferation and accumulation of malignant
cells); terminal blast phase- survival of only 3-6 months
Accelerated phase: Splenomegaly most common finding; Liver enlargement occurs but liver
function rarely affected; hyperuricemia is common and produces gouty arthritis; infection, wt
loss, fever common;
Acute effects of CML resemble acute leukemia but more prominent and painful splenomegaly.
Eval/Tx:
Dx CLL: detection of monoclonal B cell lymphocytosis of blood
Typically CLL survives 10 years or more
Multiple Myeloma p. 983
A clonal plasma cell cancer characterized by slow proliferation of tumor cell masses in bone
marrow associated with lytic bone lesions
Patho- Genetic mutations affect different pathways altering intrinsic biology of the plasma cell
generating the features of myeloma. These genetic mutations affect genes that impact cell
growth & divisions causing excessive proliferations of plasma cells.
Clinical manifestations:
Elevate levels of Ca in blood, renal failure, anemia, bone lesions.
Hypercalcemia and bone lesions result from infiltration of the bone by malignant plasma cells
and stimulation of osteoclasts to reabsorb bone. This process results in the release of Ca
Infectious Mononucleosis p. 966
A benign, acute, self-limiting lymphoproliferative clinical syndrome characterized by acute viral
infection of B lymphocytes (B cells). Associated with several tumors, most commonly specific
lymphomas and nasopharyngeal carcinoma. Most common cause- EBV.
Major manifestations of EBV induced IM: Pharyngitis, lymphadenopathy, Fever.
Other viral causes for IM: CMV, adenovirus, HIV, Hep A, Influenza A&B, Rubella.
Transmission of EBV: Saliva (Kissing Disease), secretions of genitalia rectum resp tract & blood.
No aerosol transmission.
Disease begins with widespread infection of B lymphocytes which have receptors for EBV. Virus
initially infects oropharynx, nasopharynx, and salivary epithelial cells then spreads to lymphoid
tissue and B cells. Infection of B cells allows the virus to enter the bloodstream, then the virus
spreads systemically.
Patho of Infectious Mononucleosis p. 966
In the immunocompetent patient, unaffected B cells produce antibodies (IgG, IgM, IgA) against
the virus. There is a massive activation of proliferation of cytotoxic T cells (CD8) directed against
EBV infected cells. Immune response against EBV is largely responsible for cellular proliferation
in the lymphoid tissue (lymph nodes, spleen, tonsils, liver). Sore throat and fever are the earliest
manifestations d/t inflammation at the site of viral entry and initial infection, usually the mouth
and throat.
Complications of infection Mononucleosis p. 967 fig 30.1
Lymphadenitis
hepatitis
Splenitis
Pneumonitis
Meningitis
Encephalitis
Clinical manifestations of infectious mononucleosis p. 967
Incubation period: 30-50 days (4-8 weeks), then a 3-5 day prodrome of fever, malaise,
arthralgias (joint pain). These symptoms vary in severity for the next 7-20 days.
At time of dx the patient has a classic triad of symptoms: fever, pharyngitis, lymphadenopathy of
cervical lymph nodes.
Pharyngitis: whitish, greyish green thick exudate.
,Severe complications: meningitis, encephalitis, guillain barre syndrome, bell's palsy, optic
neuritis, mental impairment, transverse myelitis, cerebellar ataxia, demyelinating disease.
Ocular manifestations: eyelid/periorbital edema, dry eyes, keratitis, uveitis, conjunctivitis,
retinitis, oculoglandular syndrome, choroiditis, papillitis, ophthalmoplegia. In child: Reye
syndrome
Pulmonary involvement: RARE- hilar and mediastinal lymphadenopathy, interstitial pneumonitis,
pleural effusions, pneumonia and resp fail in immunocompromised patients.
Older patient with 2 weeks of temp that can't be explained EBV should be suspected
Eval and Tx of infectious mononucleosis p. 967
Children present w/: fever, sore throat, lymphadenitis,
Young adults present w/: malaise, fatigue, lymphadenopathy and fever of unknown origin.
Blood contains increased # of atypical lymphocytes.
Dx based on Hoagland's criteria: 50% lymphocytes, 10% atypical lymphocytes in the blood with
presence of fever, pharyngitis, adenopathy confirmed by a + serologic test.
Serological test: heterophile antibodies, Monospot test (limited bc CMV, adenovirus,
toxoplasmosis also produce heterophilic antibodies causing false +).
Tx: IM is usually self limiting and intervention is rarely required. Rest & alleviation of symptoms.
No ASA used with child or adolescent d/t reye syndrome.
Pharyngitis of Strep origin tx w/ PCN or erythromycin. NO ampicillin (causes rash in patients
with IM)
Avoid strenuous activities. Steroids only with severe complications. Acyclovir with
immunocompromised.
Leukemia p. 968
A clonal malignant disorder of leukocytes in the bone marrow and usually but not always of the
blood. Common feature is an uncontrolled proliferation of malignant leukocytes causing an
overcrowding of bone marrow and decreased production of functional normal hematopoietic
cells. Thus it has been termed as an accumulation disorder as well as proliferation disorder.
Acute Leukemia p. 968
Characterized by undifferentiated or immature cells, usually a blast cell, and the onset of
disease is abrupt and rapid with a short survival time.
Chronic Leukemia p. 968
The predominant cell is more differentiated but does not function normally, with a relatively slow
progression
4 types of leukemia p. 968
,Acute Lymphocytic (ALL)
Acute Myelogenous (ALM)
Chronic Lymphocytic (CLL)
Chronic Myelogenous (CML)
Acute Leukemia p. 970
ALL- an aggressive fast growing leukemia with too many lymphoblasts (immature WBC) found
in the blood and bone marrow.
ALM- Aggressive fast growing leukemia with an excessive number of myeloblasts (immature
WBC that are not lymphoblasts) found in bone marrow and blood.
Acute leukemia is a result of B and T cell origin
ALL of B cell origin occurs mainly in children involving the mutation required for B cell
development (ETV6 & RUNX1).
ALL of T cell origin- Mutation in NOTCH1
ALL- defined by > 30% of lymphoblasts in bone marrow or blood
ALM- Epigenome alteration; incidence peaks in 6th decade of life
Clinical Manifestations:
ALL- w/in days to a few weeks stormy onset:
S/S generally the same with all acute leukemias:
Table 30.2
Fatigue from bone marrow depression, bleeding from thrombocytopenia, fever from infection.
Infection is usually gram-negative bacilli: E Coli, Pseudomonas aeruginosa, Klebsiella
pneumoniae.
Liver, spleen and lymph node enlargement more common in ALL
CNS S/S: HA, V, papilledema, facial palsy, blurred vision, auditory disturbances, meningeal
irritation
Prophylaxis is key with CNS involvement: radiation/chemo but not needed with ALM bc only 5%
of ALM have CNS involvement
Eval/Tx:
dx made by exam of blood cells and bone marrow.
Blood smear will show low RBC/PLt count and presence of leukemic blast cells.
Bone marrow will show 60-100% blast cells
Chemotherapy is Tx of choice
Supportive measures: Blood transfusion, ATB, antifungals, antivirals.
Stem Cell transplant
ATRA- made in the body from Vit A and helps cells grow and differentiate. It is a
chemotherapeutic agent used for tx.
The 5 year survival rate for ALL has increased to 65% in adults and 85% in children since 1970.
The Philadelphia chromosome is a poor prognostic indicator.
Myelosuppression is a consequence of leukemia and tx. requires hematologic support with
blood products.
, Chronic Leukemia p. 973
CLL- Slow growing Ca in which too many immature lymphocytes are found mostly in the blood
and bone marrow.
Chronic leukemia has a longer life expectancy of several years after dx.
Cause of CML is exposure to ionizing radiation.
The Philadelphia chromosome is present in 95% of CML and presence of BCR-ABL1 protein is
responsible for initiation of CML.
CLL- malignant transformation and progressive accumulation of B lymphocytes
CLL cells that accumulate in the marrow do not interfere with normal blood cell production to the
extent found in acute leukemias
Clinical Manifestations:
Lymphadenopathy, suppression of humoral immunity and increased infection with encapsulated
bacteria, < neutrophils which > risk for infection, CNS involvement rare.
CML- 3 phases of disease: Chronic phase 2-5 years symptoms not apparent; accelerated phase
6-18 months primary symptoms develop (excessive proliferation and accumulation of malignant
cells); terminal blast phase- survival of only 3-6 months
Accelerated phase: Splenomegaly most common finding; Liver enlargement occurs but liver
function rarely affected; hyperuricemia is common and produces gouty arthritis; infection, wt
loss, fever common;
Acute effects of CML resemble acute leukemia but more prominent and painful splenomegaly.
Eval/Tx:
Dx CLL: detection of monoclonal B cell lymphocytosis of blood
Typically CLL survives 10 years or more
Multiple Myeloma p. 983
A clonal plasma cell cancer characterized by slow proliferation of tumor cell masses in bone
marrow associated with lytic bone lesions
Patho- Genetic mutations affect different pathways altering intrinsic biology of the plasma cell
generating the features of myeloma. These genetic mutations affect genes that impact cell
growth & divisions causing excessive proliferations of plasma cells.
Clinical manifestations:
Elevate levels of Ca in blood, renal failure, anemia, bone lesions.
Hypercalcemia and bone lesions result from infiltration of the bone by malignant plasma cells
and stimulation of osteoclasts to reabsorb bone. This process results in the release of Ca
