1/55. Abdominal ultrasound findings in children with hemophagocytic lymphohistiocytosis.Hemophagocytic lymphohistiocytosis, a variant of histiocytosis, is characterized by an uncontrolled activation of the cellular immune system, including hepatic mononuclear phagocytic cells. Abdominal ultrasound findings in children are evaluated in this disease. We present six pediatric cases, two with familial and four with sporadic hemophagocytic lymphohistiocytosis, examined by abdominal sonography. Three signs were frequently observed: thickening of the gallbladder wall (all cases), increased periportal echogenicity (four cases), and enlarged lymph nodes in the porta hepatis (four cases). hepatomegaly, splenomegaly, and ascitic fluid may also be found. These imaging findings are not specific and may be seen in viral hepatitis. However, once hepatitis is excluded, they may suggest the diagnosis of hemophagocytic lymphohistiocytosis in a critically ill child. A bone smear must be done to establish the diagnosis.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
2/55. Occurrence of acute nonlymphoblastic leukemia in two girls after treatment of recurrent, disseminated Langerhans cell histiocytosis.The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.- - - - - - - - - - ranking = 0.625keywords = histiocytosis (Clic here for more details about this article) |
3/55. listeria monocytogenes and recurrent mycobacterial infections in a child with complete interferon-gamma-receptor (IFNgammaR1) deficiency: mutational analysis and evaluation of therapeutic options.We describe the history of a girl with interferon-gamma-receptor (IFNgammaR1) deficiency and studies performed to identify the molecular and clinical characteristics of this recently discovered disorder. This is the first report of a child from Northern europe with IFNgammaR1 deficiency. The patient, now 7 years old, first presented with swelling and reddening at the Bacille Calmette-Guerin (BCG) vaccination site, swelling of lymph nodes, hepatomegaly, and an unusually severe varicella rash at the age of 4 months. At that time, she was diagnosed with BCG histiocytosis without typical granuloma formation and was treated with antituberculous agents. During the clinical course of her illness, several different types of atypical mycobacteria and (for the first time in an IFNgammaR1-deficient patient) listeria monocytogenes were detected. Flow cytometric analysis showed that the patient's monocytes could not bind a monoclonal antibody specific for the IFNgamma-receptor. Our analysis of mRNA derived from the alpha-chain (IFNgammaR1) gene of this receptor revealed deletions of 173 bp and 4 bp in cDNA sequences originating from individual alleles. The 173 bp deletion was located between nucleotide positions 200 and 372, exactly matching those of exon 3, and the 4 bp deletion was located between nucleotide positions 561 and 564 of the coding region of the cDNA. Analysis of genomic dna revealed the presence of a G to T transition at the 5'end of the splice consensus sequence of intron 3, which explains the absence of exon 3. The other allele carried the 4-base-pair deletion (ACTC) at nucleotide positions 15-18 of exon 5. Twelve months after an allogeneic bone marrow transplantation, the patient had clinically improved.- - - - - - - - - - ranking = 0.125keywords = histiocytosis (Clic here for more details about this article) |
4/55. Recurrent viral associated hemophagocytic syndrome in a child with Langerhans cell histiocytosis.Langerhans cell histiocytosis (LCH) with subsequent viral-associated hemophagocytic syndrome (VAHS) or secondary hemophagocytic lymphohistiocytosis (HLH) is extremely rare. A 15-month-old girl with disseminated LCH experienced three episodes of VAHS during maintenance therapy. Viral infection, with influenza A, herpes simplex, and adenovirus, respectively, was documented at each episode. She recovered each time after interruption of maintenance therapy. The occurrence of fever and pancytopenia in patients with chemotherapy-treated LCH can be associated with VAHS and not with relapsing LCH.- - - - - - - - - - ranking = 0.75keywords = histiocytosis (Clic here for more details about this article) |
5/55. histiocytosis X of the larynx.The present report is concerned with the observation of histiocytosis X in the larynx in a man aged 57 years. This localisation of the process is quite unusual and we have found only one similar case in the literature. The differential diagnosis and possible etiological factors are briefly discussed.- - - - - - - - - - ranking = 0.125keywords = histiocytosis (Clic here for more details about this article) |
6/55. Marginal corneal infiltrates: a possible new manifestation of sinus histiocytosis with massive lymphadenopathy.PURPOSE: To describe unilateral deep marginal corneal infiltrates and chronic anterior uveitis in a young patient with sinus histiocytosis and massive lymphadenopathy. methods: A 20-year-old man with a painless enlargement of his cervical lymph nodes was followed for five years after debulking of these nodes on the right side for right chronic anterior uveitis and deep marginal corneal infiltrates. RESULTS: Histological sections of cervical lymph node confirmed the diagnosis of sinus histiocytosis with the typical emperipolesis. The uveitis partially responded to topical corticosteroid treatment but tended to recur upon its withdrawal. The marginal corneal infiltrates resolved gradually over a period of five years. CONCLUSIONS: Marginal corneal infiltrates and uveitis may presumably be associated with sinus histiocytosis. patients with sinus histiocytosis should also be screened for rare ocular involvement, such as uveitis and corneal infiltrates.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
7/55. Unusual presentation of mastoid eosinophilic granuloma in a young patient.eosinophilic granuloma is a well-recognized form of Langerhans cell histiocytosis, most commonly involving the skull bones, usually with an excellent prognosis. Recurrent and difficult to recognize osteolytic lesions of the skull are encountered only rarely. A patient with recurrent eosinophilic granuloma of the skull is reported. In spite of appropriate multimodality treatment, there were several recurrences, most recently with involvement of the mastoid process. Imaging studies revealed extensive involvement of surrounding structures with expansion of the tumor into the middle cranial fossa and slight pressure on the antero-medial portion of the temporal lobe of the brain. Despite extensive involvement, the patient had no complaints. Because of the rarity of such silent and unpredictable lesions, a systematic approach with regular CT and MRI follow-up is suggested.- - - - - - - - - - ranking = 0.125keywords = histiocytosis (Clic here for more details about this article) |
8/55. Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates.We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.- - - - - - - - - - ranking = 0.125keywords = histiocytosis (Clic here for more details about this article) |
9/55. Relapsing intracranial Rosai-Dorfman disease.Two patients presenting with recurrent visual impairment due to relapsing intracranial Rosai-Dorfman disease are described. In both patients a preoperative diagnosis of meningioma was made. Histological examination disclosed the characteristic picture of S100 and CD68 positive histiocytosis with prominent lymphophagocytosis. In both patients complete tumour removal by surgery was impossible with residual tissue being the origin of relapsing disease. Low dose radiation led to partial recovery of vision and resolution of the intracranial mass. review of the literature on intracranial Rosai-Dorfman disease leads to the suggestion that postoperative radiotherapy may be advisable in all cases.- - - - - - - - - - ranking = 0.125keywords = histiocytosis (Clic here for more details about this article) |
10/55. Recurrent crystal-storing histiocytosis of the lung in a patient without a clonal lymphoproliferative disorder.A 54-year-old woman developed an asymptomatic solitary lung mass, which recurred 10 years after resection. Both lesions consisted of a localized collection of intra-alveolar and interstitial macrophages filled with numerous eosinophilic cytoplasmic crystals. The crystals were non-birefringent, periodic acid-Schiff-negative, and showed polytypic reaction with both kappa and lambda light chains, and alpha, mu, and gamma heavy chains. No immunoglobulin gene rearrangements were detected. This lesion is consistent with crystal-storing histiocytosis, which has been reported in association with plasmacytoma. However, in this case, the absence of monotypic immunoglobulin staining within the crystals or the surrounding plasma cells, the absence of dna rearrangements, and the long asymptomatic course raise the possibility that this lesion may be reactive. Similar lesions are seen in the lungs of mice with immune defects. Crystal-storing histiocytosis may represent a general reaction pattern to excess immunoglobulin.- - - - - - - - - - ranking = 0.75keywords = histiocytosis (Clic here for more details about this article) |
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