Autoimmune lymphoproliferative syndrome differential diagnosis: Difference between revisions
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
Differentiating diagnosis of Lymphoma Symptoms Signs Diagnosis Additional Findings | {| class="wikitable" | ||
Fever Rash Diarrhea Abdominal pain Weight loss Painful lymphadenopathy Hepatosplenomegaly Arthritis Lab Findings | ! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating diagnosis of Lymphoma | ||
Autoimmune lymphoproliferative syndrome | ! colspan="5" style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptoms | ||
Lymphoma + – – + + – + – | ! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Signs | ||
Brucellosis + + – + + + + + Relative lymphocytosis Night sweats, often with characteristic smell, likened to wet hay | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis | ||
Typhoid fever + + – + – – + + | ! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Additional Findings | ||
Malaria + – + + – – + + Microcytosis, | |- | ||
elevated | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Fever | ||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Rash | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Diarrhea | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Abdominal pain | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Weight loss | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Painful lymphadenopathy | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Hepatosplenomegaly | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Arthritis | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Lab Findings | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Autoimmune lymphoproliferative syndrome | |||
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| | |||
| | |||
| | |||
| | |||
| | |||
| | |||
| | |||
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|- | |||
| style="background:#DCDCDC;" align="center" + |[[Lymphoma]] | |||
| + | |||
|– | |||
|– | |||
| + | |||
| + | |||
|– | |||
| + | |||
|– | |||
|Increase [[ESR]], increased [[LDH]] | |||
|[[Night sweats]], constant fatigue | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Brucellosis]] | |||
| + | |||
| + | |||
|– | |||
| + | |||
| + | |||
| + | |||
| + | |||
| + | |||
|[[Lymphocytosis|Relative lymphocytosis]] | |||
|[[Night sweats]], often with characteristic smell, likened to wet hay | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Typhoid fever]] | |||
| + | |||
| + | |||
|– | |||
| + | |||
|– | |||
|– | |||
| + | |||
| + | |||
|Decreased [[hemoglobin]] | |||
|Incremental increase in temperature initially and than sustained [[fever]] as high as 40°C (104°F) | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Malaria]] | |||
| + | |||
|– | |||
| + | |||
| + | |||
|– | |||
|– | |||
| + | |||
| + | |||
|Microcytosis, | |||
elevated [[LDH]] | |||
|"Tertian" fever: paroxysms occur every second day | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Tuberculosis]] | |||
| + | |||
| + | |||
|– | |||
| + | |||
| + | |||
| + | |||
|– | |||
| + | |||
|Mild normocytic [[anemia]], [[hyponatremia]], and | |||
[[hypercalcemia]] | |||
|[[Night sweats]], constant fatigue | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Mumps]] | |||
| + | |||
|– | |||
|– | |||
|– | |||
|– | |||
| + | |||
|– | |||
|– | |||
|[[Lymphocytosis|Relative lymphocytosis]], serum [[amylase]]<nowiki/>elevated | |||
|[[Parotid gland|Parotid]]<nowiki/>swelling/tenderness | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Rheumatoid arthritis]] | |||
|– | |||
| + | |||
|– | |||
|– | |||
|– | |||
|– | |||
|– | |||
| + | |||
|[[ESR]] and [[CRP]] elevated, positive [[rheumatoid factor]] | |||
|Morning stiffness | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[SLE]] | |||
|– | |||
| + | |||
|– | |||
| + | |||
| + | |||
|– | |||
|– | |||
| + | |||
|[[ESR]] and [[CRP]] elevated, positive [[ANA]] | |||
|[[Fatigue]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Human Immunodeficiency Virus|HIV]] | |||
|– | |||
|– | |||
|– | |||
| + | |||
| + | |||
| + | |||
|– | |||
| + | |||
|Leukopenia | |||
|Constant fatigue | |||
|} | |||
{| class="wikitable" | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Disease | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating signs and symptoms | |||
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating tests | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Autoimmune lymphoproliferative syndrome | |||
| | |||
| | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Lymphoma|CNS lymphoma]] | |||
| | |||
*Patient is [[immunocompetent]] | |||
*Focal symptoms indicative of a mass [[lesion]] | |||
*[[Seizure]] | |||
| | |||
*Single solitary ring enhancing [[lesion]] on [[CT]] or [[MRI]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Disseminated tuberculosis]] | |||
| | |||
*Prior history of residence in an [[Endemic (epidemiology)|endemic]] area | |||
*Chronic [[cough]], [[weight loss]], [[hemoptysis]] | |||
| | |||
*[[PCR]] of [[CSF]] for [[tuberculosis]] | |||
*Mycobacterial culture of [[CSF]] | |||
*[[Brain]] biopsy for [[acid-fast bacilli]] staining | |||
*Culture and acid stain positive for [[acid-fast bacilli]] | |||
*CXR shows [[Cavitation|cavitations]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Aspergillosis]] | |||
| | |||
*[[Pulmonary]] [[lesions]] in addition to [[CNS]] [[lesions]] | |||
*Symptoms may include [[cough]], [[chest pain]], and [[hemoptysis]] | |||
| | |||
*[[CSF]] fungal culture, [[galactomannan]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Cryptococcosis]] | |||
| | |||
*Symptoms include [[cough]], [[chest pain]], and [[hemoptysis]] | |||
| | |||
*[[Cryptococcal infection|Cryptococcal]] [[antigen]] from [[CSF]] and [[serum]] | |||
*[[CSF]] fungal culture | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Chagas disease]] | |||
| | |||
*History of residence in Central or South America | |||
*Acute infection is rarely symptomatic | |||
*[[Encephalitis]] or focal [[brain]] [[lesions]] | |||
*[[Myocarditis]] | |||
*[[Chronic]] [[infections]] in [[immunocompromised]] patients develop into [[encephalitis]] with [[necrotic]] [[brain]] lesions causing a [[mass effect]] | |||
| | |||
*[[Trypanosoma cruzi]] in [[blood]], [[Tissue (biology)|tissue]], or [[CSF]], [[PCR]] of [[Tissue (biology)|tissue]] or [[body fluids]], and [[Serological testing|serologic tests]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Cytomegalovirus infection|CMV infection]] | |||
| | |||
*Most common [[CNS]] [[opportunistic infection]] in [[AIDS]] patients | |||
*Presents with [[encephalitis]], [[retinitis]], progressive [[myelitis]], or [[polyradiculitis]] | |||
*In [[disseminated disease]], it involves both the [[liver]] and kidneys | |||
| | |||
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually near the [[brain stem]] or periventricular areas | |||
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic | |||
*[[Brain biopsy]] with + [[staining]] for [[CMV]] or evidence of owl's eyes is also diagnostic, but it is rarely performed because of the location of [[brain]] lesions | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[HSV|HSV infection]] | |||
| | |||
*[[Seizures]], [[headache]], [[confusion]] and/or [[urinary retention]] can be seen in [[disseminated disease]], which usually affects only the [[immunocompromised]] or acute [[infections]] | |||
*In [[pregnant]] women, it may be associated with concurrent [[genital]]/[[oral]] [[lesions]]; can be spread to the [[neonate]] during acute infection in the mother, or via [[viral shedding]] in the [[birth canal]] | |||
*[[Neonatal]] [[Herpes simplex virus|HSV]] can range from localized [[Skin and soft-tissue infections|skin infections]] to [[encephalitis]], [[pneumonitis]], and [[disseminated disease]] | |||
| | |||
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually the [[medial]] [[temporal lobe]] or the [[Orbital cavity|orbital]] surface of the [[frontal lobe]]. | |||
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Chickenpox|Varicella Zoster infection]] | |||
| | |||
*Multifocal involvement has subacute course, usually only in [[immunosuppressed]], with [[headache]], [[fever]], focal deficits, and [[seizures]]. | |||
*Unifocal involvement is more typically seen in [[immunocompetent]] hosts, occurring after [[contralateral]] [[cranial nerve]] [[herpes zoster]], with [[Altered mental status|mental status changes]], [[TIA|TIAs]], and [[stroke]] | |||
*[[Disseminated disease|Disseminated]] [[varicella zoster virus]] can occur in adults during primary [[infection]], presenting with [[pneumonitis]] and/or [[hepatitis]] | |||
*Disease is a [[Vasculitis|vasculopathy]] with [[hemorrhage]] and [[stroke]] | |||
| | |||
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Brain abscess]] | |||
| | |||
*Associated with [[sinusitis]] (abutting the sinuses) or with [[bacteremia]] | |||
*Signs and symptoms includes [[fever]] and [[necrotizing]] [[brain]] [[lesions]] with [[mass effect]] | |||
| | |||
*[[CSF]] culture or culture of [[brain abscess]] | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |[[Progressive multifocal leukoencephalopathy]] | |||
| | |||
*Symptoms are often more insidious in onset and progress over months. Symptoms include progressive [[weakness]], poor [[coordination]], with gradual slowing of [[mental]] function. Only seen in the [[immunosuppressed]]. Rarely associated with [[fever]] or other systemic symptoms | |||
| | |||
*[[Polymerase chain reaction|PCR]] of [[CSF]] for [[JC virus]] | |||
*[[Biopsy]] reveals [[white matter]] [[lesions]] and not well-circumscribed [[lesions]]. | |||
|} | |||
PCR of CSF for JC virus | |||
Biopsy reveals white matter lesions and not well-circumscribed lesions. | |||
==References== | ==References== |
Revision as of 22:41, 1 July 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Due to having overlapping presenting symptoms with other hematologic disorders, Autoimmune lymphoproliferative syndrome in children should be excluded from infection, autoimmune disease, inherited immune disorders, and lymphoma.
Differential Diagnosis
Differentiating diagnosis of Lymphoma | Symptoms | Signs | Diagnosis | Additional Findings | ||||||
---|---|---|---|---|---|---|---|---|---|---|
Fever | Rash | Diarrhea | Abdominal pain | Weight loss | Painful lymphadenopathy | Hepatosplenomegaly | Arthritis | Lab Findings | ||
Autoimmune lymphoproliferative syndrome | ||||||||||
Lymphoma | + | – | – | + | + | – | + | – | Increase ESR, increased LDH | Night sweats, constant fatigue |
Brucellosis | + | + | – | + | + | + | + | + | Relative lymphocytosis | Night sweats, often with characteristic smell, likened to wet hay |
Typhoid fever | + | + | – | + | – | – | + | + | Decreased hemoglobin | Incremental increase in temperature initially and than sustained fever as high as 40°C (104°F) |
Malaria | + | – | + | + | – | – | + | + | Microcytosis,
elevated LDH |
"Tertian" fever: paroxysms occur every second day |
Tuberculosis | + | + | – | + | + | + | – | + | Mild normocytic anemia, hyponatremia, and | Night sweats, constant fatigue |
Mumps | + | – | – | – | – | + | – | – | Relative lymphocytosis, serum amylaseelevated | Parotidswelling/tenderness |
Rheumatoid arthritis | – | + | – | – | – | – | – | + | ESR and CRP elevated, positive rheumatoid factor | Morning stiffness |
SLE | – | + | – | + | + | – | – | + | ESR and CRP elevated, positive ANA | Fatigue |
HIV | – | – | – | + | + | + | – | + | Leukopenia | Constant fatigue |
Disease | Differentiating signs and symptoms | Differentiating tests |
---|---|---|
Autoimmune lymphoproliferative syndrome | ||
CNS lymphoma |
|
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Disseminated tuberculosis |
|
|
Aspergillosis |
|
|
Cryptococcosis |
|
|
Chagas disease |
|
|
CMV infection |
|
|
HSV infection |
|
|
Varicella Zoster infection |
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Brain abscess |
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Progressive multifocal leukoencephalopathy |
|