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Coexpression of CD1a, langerin and Birbeck's granules in Langerhans
cell histiocytoses (LCH) in children: ultrastructural and
immunocytochemical studies.Folia
Histochem Cytobiol. 2007;45(1):21-5.
Langerhans cell
histiocytoses (LCH) represent rare diseases of unclear etiology and
pathogenesis. Most of the cases include children, 1 to 15 years of
age, and various organs are involved (bones, skin, liver, lymph nodes,
bone marrow and other). The diagnosis of LCH used to be established by
biopsy of the inflamed tissue and demonstration of expression of
markers specific for Langerhans cells: CD1a and langerin. The
diagnosis can be ultimately confirmed by demonstration of Birbeck's
granules in the electron microscopy. The present study was aimed at
immunocytochemical demonstration, in the examined LCH material (skin,
bones, lymph nodes), of the specific antigen expression and at
comparing it with the presence of Birbeck's granules. In the examined
11 cases co-expression of CD1a with langerin and with the presence of
Birbeck's granules was noted. Also in all examined biopsies the
expression of S-100 protein on inflammatory cells was found. The
results corroborate the usefulness of immunocytochemical studies on CD
1 a and langerin expression in diagnosis of LCH.
Solitary
intracerebral langerhans cell histiocytosis: report of one case and
literature review. Rev Med
Interne. 2007 May 25.
INTRODUCTION:
Langerhans cell histiocytosis is a multivisceral pathology.
Neurological manifestations are rare. EXEGESIS: We report the case of
a 31 year old man hospitalized for left partial motor seizure
revealing a right frontal tumor with criteria for histiocytosis X. The
histological and biological examination found criteria for Langerhans
cell histiocytosis (CD1a and S100 reactivity). The check-up for
extracerebral localisations of the disease was negative. The outcome
was favourable after a total surgical resection. The review of the
literature and a discussion on neurological manifestations of this
disease were carried out. CONCLUSION: A neurological manifestation can
be the first and only symptom of a Langerhans cell histiocytosis.
Langerhans
cell histiocytosis of vulva in adolescent.Int
J Gynecol Cancer. 2007
Mar-Apr;17(2):520-4.
Langerhans cell histiocytosis (LCH) affecting child vulva alone is a
very rare disease. Only 13 cases of primary vulvar LCH have been
previously reported in the medical literature. We describe an
additional case in which the LCH was confined to the vulva, with
review of the literature. A 16.5-year-old girl presented with papulous
and ulcerative lesions on her labia majora and minora. The biopsy
revealed a typical histopathologic finding consistent with LCH. A
metastatic work-up did not reveal any evidence of the disease except
on the vulva. Treatment was carried out according to LCH II protocol.
The patient was diagnosed with a recurrent disorder in the vulva 8
months after the completion of primary chemotherapy. For this reason,
she underwent second line treatment with 2-chlorodeoxyadenosine.
Eighteen months after the second line chemotherapy, the patient has no
signs of a local or systemic recurrence. Primary LCH of vulva is very
unusual, but we have to keep in mind this possibility when an
adolescent girl presents with an atypical chronic lesion on the vulva.
This patient appears to be the first case of adolescent 16.5 year old
having a solely cutaneous lesion of the vulva.
Pulmonary langerhans cell
histiocytosis (histiocytosis X) on bronchoalveolar lavage: a report of
2 cases.Acta
Cytol. 2007 May-Jun;51(3):480-2.
BACKGROUND:
Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial lung
disease characterized by bilateral nodular and cystic lesions.
Clinically it seems to be a reactive process related to cigarette
smoking. CASES: In 2 cases of PLCH, cytologic and immunocytochemical
evaluation of bronchoalveolar lavage (BAL) fluid was successfully used
for the diagnosis of PLCH. Two heavy smokers complained of fever,
cough and debilitation. Serologic and hematologic values were normal.
In both cases radiography and computed tomography (CT) were similar,
showing multiple bilateral nodular or cystic lesions in the middle and
upper lung zones. Cytospins obtained from BAL were Papanicolaou and
May-Grünwald-Giemsa stained; others were immunostained with
cytokeratin cocktail, CD1a and S-100. Cytospins showed a monomorphous
and dispersed cell population consisting ofmononucleated or
binucleated and occasionally multinucleated histiocytes. Single cells
showed wide, well-defined, acidophilic cytoplasm and oval or
kidney-shaped, vesicular nuclei with irregular shapes, evident
nucleoli and frequent grooves and indentations. Immunocytochemical
staining showed diffuse cytoplasmic positivity for S-100 and CD1a and
negativity for cytokeratin; only the few cylindrical cells present in
the cytospins were positive for cytokeratin. In both cases the
cytologic diagnosis of PLCH was confirmed by subsequent CT and
clinical follow-up. CONCLUSION: Cytologic and immunocytochemical
evaluation of BAL fluid permits a definitive diagnosis of PLCH. This
cytologic diagnosis is clinically relevant because it permits surgical
biopsy to be bypassed and allows waiting for a possible spontaneous or
pharmacologic resolution.
Immunohistochemical analysis of langerin in langerhans cell
histiocytosis and pulmonary inflammatory and infectious diseases.Am
J Surg Pathol. 2007 Jun;31(6):947-52.
Pulmonary
Langerhans cell histiocytosis (LCH) is an idiopathic condition
affecting predominantly adult smokers. Histologically, LCH is
characterized by a nodular, interstitial proliferation of Langerhans
cells around the distal airways with associated eosinophils,
lymphocytes, and macrophages. Associated findings, such as fibrosis,
emphysematous change, and bronchiolitis can be reminiscent of other
interstitial lung diseases. The markers CD1a and S100 have
traditionally been used to distinguish LCH from other processes.
Little is known about expression of the Langerhans cell-specific
lectin, langerin, in pulmonary diseases. We examined the expression
patterns of S100, CD1a, and langerin in LCH and other interstitial,
inflammatory, and infectious processes in cases retrieved from the
files at Brigham and Women's Hospital Department of Pathology.
Immunoreactivity was scored according to the number of cells staining
per high power field (400x) in areas of highest density, averaged over
4 fields. Cases diagnosed as LCH based on histomorphology and positive
CD1a and S100 staining demonstrated strong langerin positivity in
lesional tissue. All cases of LCH contained greater than 30 langerin
and CD1a positive cells per high power field (HPF), with a mean of
>100 cells per HPF, in lesional tissue. Of the other interstitial
processes examined, only usual interstitial pneumonia demonstrated
increased number of Langerhans cells within epithelium and
interstitium (mean 14 cells per HPF) as compared with normal lung
(mean 6 cells per HPF). Langerin and CD1a serve as specific diagnostic
markers in distinguishing LCH from other interstitial and inflammatory
processes.
A case of
Langerhans cell histiocytosis presented with pneumothorax.J
Pediatr Hematol Oncol. 2007
Jan;29(1):60-2.
Pneumothorax (PTX) is an unusual complication of Langerhans cell
histiocytosis (LCH) in childhood. Spontaneous PTX is rare in
childhood, and it is very rare in infancy. There are no specific
recommendations for the treatment of PTX from LCH described in the
literature. We are presenting a 19-month-old boy, who suddenly
developed left-sided PTX with infiltrations in both lungs. He
presented with PTX and skin lesions. He had a prolonged cardiac
arrest, and although resuscitation was successful he required
continuing ventilatory support (intermittent positive-pressure
ventilation). Because he suddenly developed right-sided PTX and died
on the second day of the admission, his LCH diagnosis was made only
postmortem. So, he did not receive chemotherapy. It is likely that
intermittent positive-pressure ventilation during the operation
induced the development of much more multiple lung bullae, which
subsequently ruptured, and/or it facilitated the development of the
right-sided PTX. The patients with PTX and skin lesions, including
babies, most likely have LCH and specific chemotherapy should be
started in emergency, even before the final diagnosis is achieved.
Differences in telomerase
expression by the CD1a+ cells in Langerhans cell histiocytosis reflect
the diverse clinical presentation of the disease.J
Pathol. 2007 Jun;212(2):188-97.
Langerhans
cell histiocytosis (LCH) is a disease characterized by an uncontrolled
clonal proliferation of Langerhans cells, whose aetiology is still
unclear. The clonal nature of LCH could support the hypothesis that it
is a neoplastic disease with unlimited growth potential. One
requirement for unlimited proliferation is the maintenance of telomere
length. In a group of 70 patients, we set out to investigate whether a
telomere maintenance mechanism is indeed active in LCH cells. This
work showed that LCH cells from all restricted skin LCH lesions (6/6)
expressed telomerase as assessed by human telomere reverse
transcriptase (hTERT) immunohistochemistry, whereas LCH cells from the
majority of the bone lesions analysed did not express hTERT (26/34).
Interestingly, in contrast to the solitary bone lesions, LCH cells
from lesions of multi-system patients always expressed telomerase
(11/11), regardless of the lesional site. In situ telomeric repeat
amplification protocol (TRAP) assays performed on different lesional
sites showed that this telomerase was active. In addition, the
telomere length of LCH cells from a hTERT-positive skin multi-system
lesion was long and homogeneous when compared to that in the LCH cells
from hTERT-negative bone single-system LCH lesions, which was
heterogeneous in length. No evidence for an alternative lengthening of
telomeres mechanism was found in hTERT-negative lesions. The
difference in telomerase expression and telomere length at the
different lesional sites and in biopsies from patients with solitary
versus multi-system disease appears to reflect the diverse clinical
presentation and course of this disease. The results from this study
have important implications for understanding the nature of this
disease.
Langerhans
cell histiocytosis of the skull on cytologic squash preparations.
Diagn Cytopathol. 2007 Mar;35(3):154-7.
We present a
case in which a primary cytodiagnosis of Langerhans cell histiocytosis
(LCH) of the skull was made using squash preparations. The patient, a
25-year-old male, presented with raised intracranial pressure and
decreased visual acuity. Magnetic resonance imaging revealed a large
skull lesion with osteolytic features in the left frontal bone. The
patient underwent surgical resection by the extended basal frontal
epidural approach. The squash preparation smears were cellular and
demonstrated a mixed population of small, mature lymphocytes,
eosinophils, and a high histiocytes content. The histiocytes occurred
as isolated or loosely cohesive and clustered. They possessed abundant
cytoplasm with rounded cell shape and had characteristic nuclear
features, composed of fine chromatin and delicate nuclear membranes.
The cytologic features of these histiocytes were consistent with
Langerhans cells (LCs). A final impression of LCH of the skull was
rendered. Subsequent histopathology confirmed the diagnosis. LCs
reacted with both S-100 protein and CD1a immunohistochemically. The
demonstration of Birbeck granules on electron microscopic study was
also noted. Whenever squash preparation yields a mixed population of
mature lymphocytes, eosinophils, and histiocytes, the cytologists
should be aware of and consider LCH as a diagnostic possibility.
Mandibular
Langerhans cell histiocytosis.Rev
Stomatol Chir Maxillofac. 2006
Dec;107(6):465-9.
INTRODUCTION: Langerhan's cell histiocytosis is defined as an abnormal
proliferation of Langerhans cells in various organs and tissues (bone,
skin, lymph nodes). Among facial locations, mandibular involvement is
the most frequent and occurs in young people less than 20 years. The
aim of this report is to review the characteristic features of this
disease, the current means of diagnosis and treatment through two case
reports.CASES: Two male patients aged 10 and 55 years respectively
presented with single mandibular location of Langerhans cell
histiocytosis. The diagnosis was confirmed by histological examination
of the biopsy specimen. The first patient underwent surgical resection
of the tumor. No recurrence was observed. In the second patient, the
postoperative course was marked by the appearance of an inflammatory
phenomenon in the adjacent skin and mucosa, successfully treated by
intralesional corticosteroid therapy.DISCUSSION: Langerhan's cell
histiocytosis is a rare disease. The mandibular location has variable
clinical expressions which can vary from dental mobility to tumor
development in the gingiva. The radiological appearance is also
variable; histological proof is required for diagnosis. Treatment
depends on the number and locations of the lesions. In case of a
single location, the treatment options may vary from a "wait and see"
attitude to a non aggressive surgery, with a possible local
corticosteroid therapy or radiothe-rapy. For multiples locations,
chemotherapy is indicated.
Biology of Langerhans
cells and Langerhans cell histiocytosis.Int
Rev Cytol. 2006;254:1-43.
Langerhans
cells (LC) are epidermal dendritic cells (DC). They play an important
role in the initiation of immune responses through antigen uptake,
processing, and presentation to T cells. Langerhans cell histiocytosis
(LCH) is a rare disease in which accumulation of cells with LC
characteristics (LCH cells) occur. LCH lesions are further
characterized by the presence of other cell types, such as T cells,
multinucleated giant cells (MGC), macrophages (MPhi), eosinophils,
stromal cells, and natural killer cells (NK cells). Much has been
learned about the pathophysiology of LCH by studying properties of
these different cells and their interaction with each other through
cytokines/chemokines. In this review we discuss the properties and
interactions of the different cells involved in LCH pathophysiology
with the hope of better understanding this enigmatic disorder.
Oral
manifestations of langerhans cell histiocytosis in a pediatric
population: a clinical and histological study of 8 patients.J
Craniofac Surg. 2006 May;17(3):552-6.
Langerhans
Cell Histiocytosis is a rare and protean disease of unknown etiology,
clinically characterized by single or multiple osteolytic and/or soft
tissue lesions. Oral involvement may predate systemic manifestations
or mimic infectious/inflammatory disorders. In the present series,
oral involvement characterized eight cases of pediatric Langerhans
Cell Histiocytosis and oral symptoms represented a very early sign of
this disease in four patients. A careful clinical investigation and a
proper histologic analysis were required for achieving a definite
diagnosis and for confirming clinical suspicion of disease relapse.
Immunohistochemistry, that nowadays represents a reliable and
accessible ancillary histological technique, allowed the definite
diagnosis in all cases of our series.
Liver
involvement in Langerhans' cell histiocytosis. Case report.J
Gastrointestin Liver Dis. 2006
Mar;15(1):57-9.
Langerhans'cell histiocytosis (Histiocytosis X) is a rare disease of
unknown cause characterized by oligoclonal proliferation of Langerhans
cells. It occurs mostly in children and young adults and involves one
or more body systems such as bone, hypothalamus, posterior pituitary
gland, lymph nodes, liver or various soft tissues.The diagnosis is
always made by a histological approach.We report a case of
Langerhans'cell histiocytosis in a young patient with clinical signs
of diabetes insipidus and hepatic involvement in whom the
immunohistochemical analysis of the liver tissue led to the definitive
diagnosis.
Multiple eyelid defects in
cutaneous Langerhans cell histiocytosis.Ophthal
Plast Reconstr Surg. 2006
May-Jun;22(3):216-8.
Langerhans
cell histiocytosis is a disorder characterized by the proliferation of
histiocytes of the mononuclear phagocyte system. Cutaneous Langerhans
cell histiocytosis can develop as a solitary lesion or more frequently
as part of a multisystem disease. This report describes a case of
cutaneous Langerhans cell histiocytosis affecting the eyelids,
resulting in a coloboma-like defect. The clinical features and
management are discussed.
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