Autoimmune lymphoproliferative syndrome differential diagnosis

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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 hypercalcemia

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 Patient is immunocompetent Focal symptoms indicative of a mass lesion Seizure Single solitary ring enhancing lesion on CT or MRI Disseminated tuberculosis Prior history of residence in an 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 cavitations Aspergillosis Pulmonary lesions in addition to CNS lesions Symptoms may include cough, chest pain, and hemoptysis CSF fungal culture, galactomannan Cryptococcosis Symptoms include cough, chest pain, and hemoptysis Cryptococcal antigen from CSF and serum CSF fungal culture 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, or CSF, PCR of tissue or body fluids, and serologic tests 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 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 HSV can range from localized skin infections to encephalitis, pneumonitis, and disseminated disease Brain CT/MRI/biopsy: location of lesions is usually the medial temporal lobe or the orbital surface of the frontal lobe. PCR of CSF with detectable virus is diagnostic 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 mental status changes, TIAs, and stroke Disseminated varicella zoster virus can occur in adults during primary infection, presenting with pneumonitis and/or hepatitis Disease is a vasculopathy with hemorrhage and stroke PCR of CSF with detectable virus is diagnostic 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 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 PCR of CSF for JC virus Biopsy reveals white matter lesions and not well-circumscribed lesions.