Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, cytopenias, splenomegaly, and hemophagocytosis by macrophages activated by high cytokine levels. [ncbi.nlm.nih.gov]

DANGER SIGNS: failure to thrive, inflammatory anemia, thrombocytopenia, splenomegaly, lymphadenopathy, joint swelling/bone pain, dysmorphia, recurrent serious infections, fever/infectious symptoms every 21 days, unusual or resistant infections, periodontal [hawaii.edu]

Venopathy of the portal vein was observed in 80% and was associated with splenomegaly [ 116 ]. [clinicalmolecularallergy.biomedcentral.com]

Chronic granulomatous disease is a rare, inherited immunodeficiency disorder that reduces the superoxide generation ability of phagocytes, leading to recurrent infections and granulomatous inflammation. [ncbi.nlm.nih.gov]

Chronic granulomatous disease (CGD), a rare immunodeficiency, typically presents with recurrent infections caused by catalase-positive organisms. [scholars.northwestern.edu]

We present the case of a 15-year-old boy with X-linked CGD who presented with unremitting cough and fevers. He had a left-sided pneumonia that persisted despite home intravenous antibiotics. [ncbi.nlm.nih.gov]

This article discusses an 8-week-old Hispanic male who presented with fever of unknown origin. [pediatrics.aappublications.org]

Fevers, however, are very common and an infectious focus should always be searched for in any child presenting with a fever before attributing the fever to the disorder itself and not to an ongoing infection. [lecturio.com]

Hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. [ncbi.nlm.nih.gov]

Hypergammaglobulinemia and anemia can occur; ESR is elevated. [merckmanuals.com]

Most common presentation is a young patient with short stature, dermatitis, gingivitis, anemia of chronic disease, recurrent lymphadenopathy, hepatosplenomegaly, hepatic abscesses and multifocal, recurrent osteomyelitis. [radiopaedia.org]

[…] kills microorganisms in lysosomes Either Y linked or autosomal recessive Diagnose with nitro blue tetrazolium test (almost always positive) Clinically, patients have recurrent lymphadenitis, hepatosplenomegaly, skin rash, pulmonary edema Laboratory Anemia [pathologyoutlines.com]

Phone: 617-636-5535 Fax #: 617-636-7738 Childhood cancers including leukemia, lymphoma and solid tumors, anemia and bleeding disorders, thrombosis View Full Profile Susan K. [floatinghospital.org]

The most common side effects with ACTIMMUNE are “flu-like” symptoms such as fever, headache, chills, muscle pain, or fatigue, which may decrease in severity as treatment continues. [actimmunehcp.com]

Adverse events—including fever, rigors, flushing, vomiting, irritability, or agitation, and, in two patients, transient dyspnoea—were reported in less than 2% of 1594 individual infusions. Significantly, 16 patients developed allo-immunisation. [f1000research.com]

We report the fatal case of a 4-year-old boy with chronic granulomatous disease, who presented with sepsis after a few days of abdominal pain and diarrhea. [ncbi.nlm.nih.gov]

Common side effects of antibiotics include diarrhea and upset stomach. Infections from fungi, like Aspergillus species, can be very serious for people with CGD. [cgdconnections.com]

diarrhea ear infections osteomyelitis failure to thrive Physical exam short stature eczematoid dermatitis hepatomegaly lymphadenopathy Evaluation Flow cytometry reduction of dihydrorhodamine abnormal can detect X-linked carrier status cannot differentiate [medbullets.com]

most common clinical findings are lymphadenopathy, hypergammaglobulinemia, hepatomegaly, splenomegaly, anemia of chronic disease, underweight, chronic diarrhea, short stature, gingivitis, and dermatitis; diagnosis made by NBT, flow cytometry, or cytochrome [hawaii.edu]

In addition, septicopyemia, thrush, and hepatosplenomegaly were also commonly observed, accounting for 23%, 23%, and 40% of the cases. Lesions due to BCG vaccination occurred in more than half of the patients. [ncbi.nlm.nih.gov]

Multifocal and recurrent unusual osteomyelitis, recurrent hepatic abscesses, hepatosplenomegaly, and lymphadenitis. For lung involvement see - pulmonary manifestations of chronic granulomatous disease. [radiopaedia.org]

[…] monocytes White blood cells cannot generate superoxide ion which kills microorganisms in lysosomes Either Y linked or autosomal recessive Diagnose with nitro blue tetrazolium test (almost always positive) Clinically, patients have recurrent lymphadenitis, hepatosplenomegaly [pathologyoutlines.com]

Suppurative lymphadenitis, hepatosplenomegaly, pneumonia, and hematologic evidence of chronic infection are common. Skin, lymph node, lung, liver, and perianal abscesses; stomatitis; and osteomyelitis also occur. Growth may be delayed. [merckmanuals.com]

Since its first description in the 1950s as a syndrome of recurrent infections, hypergammaglobulinemia, hepatosplenomegaly, and lymphadenopathy in males who invariably died in the first decade of life, notable advances have been made in the understanding [thehealthscience.com]

[…] recurrent pyogenic infections recurrent infection with catalase-positive organisms pneumonias aspergillosis skin abscesses pulmonary abscesses chronic diarrhea ear infections osteomyelitis failure to thrive Physical exam short stature eczematoid dermatitis hepatomegaly [medbullets.com]

lymphadenopathy or hepatomegaly) is “normal” for these patients. These findings usually indicate that there is significant pathology ongoing and it is important to make a definitive diagnosis and treat accordingly. [clinicaladvisor.com]

most common clinical findings are lymphadenopathy, hypergammaglobulinemia, hepatomegaly, splenomegaly, anemia of chronic disease, underweight, chronic diarrhea, short stature, gingivitis, and dermatitis; diagnosis made by NBT, flow cytometry, or cytochrome [hawaii.edu]

In one study of 194 patients from the NIH, elevated liver enzymes (mainly transaminitis) were documented in 75%, liver abscess in 35%, hepatomegaly in 34%, and splenomegaly in over 50% cases [ 116 ]. [clinicalmolecularallergy.biomedcentral.com]

Abstract Chronic lupus-like illness, characterized by arthralgia, cutaneous manifestations of lupus erythematosus, and photosensitivity, occurred in two mothers of boys with chronic granulomatous disease; one mother also had Raynaud's phenomenon, pleuritis [annals.org]

Cutaneous manifestations of chronic granulomatous disease. A report of four cases and review of the literature. J Am Acad Dermatol. 1997 Jun. 36(6 Pt 1):899-907. [Medline]. Gallin JI, Malech HL. Update on chronic granulomatous diseases of childhood. [emedicine.medscape.com]

Chronic granulomatous disease was suspected in this case after Serratia marcescens was isolated from a skin ulcer culture. Granulomas were confirmed on histology and chronic granulomatous disease was diagnosed. [ncbi.nlm.nih.gov]

The abscesses extended along the dura mater for 7 years and finally resulted in fatal meningitis and cervical myelitis. [ncbi.nlm.nih.gov]

Google Scholar Fleischmann J, Church JA, Lehrer RI: Primary Candida meningitis and chronic granulomatous disease. [clinicalmolecularallergy.biomedcentral.com]

Incidentally, a space-occupying mediastinal mass was revealed that was further evaluated by magnetic resonance imaging. Varying solid and also cystic, septated parts of the mass could be discerned and it was considered to be a teratoma. [ncbi.nlm.nih.gov]

Lesions those are contiguous with the great vessels or the pericardium, single cavitary lesion that cause hemoptysis, lesions invading the chest wall, aspergillosis that involves the skin and the bone are the indications for surgical therapy.Chronic granulomatous [ncbi.nlm.nih.gov]

It may present with recurrent pneumonia, hilar lymphadenopathy, empyema, abscess, reticulonodular patterns, and granulomas due to lung involvement. In recent years, mycobacterial disease susceptibility has been reported in CGD cases. [ncbi.nlm.nih.gov]

Species of the genus Fusarium are opportunistic environmental microorganisms that are rarely pathogenic in humans. We report here the first case of X-linked CGD complicated with epidural abscess caused by Fusarium falciforme infection. [ncbi.nlm.nih.gov]

Chronic granulomatous disease (CGD) is a life threatening inherited disorder with varied clinical presentations often characterized by recurrent bacterial and fungal infections along with widespread granulomatous tissue response. [ncbi.nlm.nih.gov]

Infection of the bones (osteomyelitis), brain abscesses, obstruction of the genitourinary tract and/or gastrointestinal tract due to the formation of granulomatous tissue, and delayed growth are also symptomatic of chronic granulomatous disease. [rarediseases.org]