Displaying publications 1 - 20 of 34 in total

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  1. Leung AKC, Lam JM, Leong KF
    World J Pediatr, 2019 Dec;15(6):536-545.
    PMID: 31456157 DOI: 10.1007/s12519-019-00304-9
    BACKGROUND: Langerhans cell histiocytosis (LCH) is a group of diseases characterized by the proliferation and accumulation of Langerhans cells. Clinical presentations of LCH vary widely.

    DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.

    RESULTS: Generally, patients with LCH can be divided into two groups based on the extent of involvement at diagnosis, namely, single-system LCH and multisystem LCH. The involvement may be unifocal or multifocal. Patients with isolated bone lesions typically present between 5 and 15 years of age, whereas those with multisystem LCH tend to present before 5 years of age. The clinical spectrum is broad, ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition. Clinical manifestations include, among others, "punched out" lytic bone lesion, seborrheic dermatitis-like eruption, erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches, and eczematous lesions, diabetes insipidus, hepatosplenomegaly, cytopenias, lymphadenopathy, and an acute fulminant disseminated multisystem condition presenting with fever, skin rash, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. The diagnosis is clinicopathologic, based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue. Positive CD1a, S100, and/or CD207 (Langerin) immunohistochemical staining of lesional cells is required for a definitive diagnosis. Watchful waiting is recommended for patients with skin-only LCH. Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids, topical nitrogen mustard, oral methotrexate, or oral hydroxyurea. The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine. Mercaptopurine is added for patients with risk organ involvements.

    CONCLUSIONS: Because of the broad spectrum of clinical manifestations and the extreme diversity of disease, LCH remains a diagnostic dilemma. Morphological identification of LCH cells and positive immunochemical staining with CD1a, S100, and/or CD207 (Langerin) of lesional cells are necessary for a definitive diagnosis.

    Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/diagnosis*
  2. Fadilah SAW, Raymond AA, Cheong SK, Amir MAL
    Med J Malaysia, 2001 Dec;56(4):503-7.
    PMID: 12014773
    A fulminant clinical presentation with high fever and hepatosplenomegaly, together with a course of worsening pancytopenia, coagulopathy and liver failure, is suggestive of the haem syndrome (HPS). Bone marrow examination is diagnostic. We present 3 cases of HPS associated with different aetiologies including acute Ebstein Barr virus infection, T cell lymphoma, and malignant histiocytosis. In all the cases, the diagnosis was made late and the patients succumbed before definitive therapy could be administered.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/mortality*; Histiocytosis, Non-Langerhans-Cell/pathology*; Histiocytosis, Non-Langerhans-Cell/therapy
  3. Mohd Ariff S, Joehaimey J, Ahmad Sabri O, Zulmi W
    Malays Orthop J, 2011 Nov;5(3):28-31.
    PMID: 25279033 MyJurnal DOI: 10.5704/MOJ.1111.004
    Langerhans cell histiocytosis of the spine frequently presents as a solitary lesion and rarely results in neurological deficit. Involvement of thyroid tissue is also rare, even in multifocal disease presentations. We present an unusual variant of Langerhans cell histiocytosis in a 37-year-old man presenting with profound quadriparesis and incidental thyroid involvement. MR imaging and skeletal radiographs revealed widespread involvement of the spine. The patient underwent surgical resection of the tumour and stabilization of the spine. Diagnosis was confirmed by histological and immunohistochemistry analysis. Langerhans cell histiocytosis should be included in the differential diagnosis of radiolucent lesions of the spine in adults.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell*
  4. Hoh MC, Lin HP
    Med J Malaysia, 1997 Jun;52(2):146-50.
    PMID: 10968072
    Haemophagocytic Lymphohistiocytosis (HLH) is a rare clinical illness with a high mortality. There are reported effective treatment and a favourable outcome if diagnosed early. Five cases of childhood non-familial HLH seen over a 3 year period in our hospital are presented. The diagnosis was not suspected in the referring hospitals even after a bone marrow biopsy examination in two cases. Delay in referral was between 2 weeks to 2 months. A viral trigger was detected in only two cases. There were two deaths. Cause of death in both cases were cytomegalovirus pneumonitis and disseminated intravascular coagulopathy. Respond to treatment was better if started earlier. One case spontaneously resolved. Earlier diagnosis will lead to prompt treatment and a better outcome.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/diagnosis*; Histiocytosis, Non-Langerhans-Cell/therapy
  5. Ong ST, Lian CB
    J Clin Pediatr Dent, 1992;17(1):33-5.
    PMID: 1290758
    A case of chronic disseminated histiocytosis X (Hand-Schuller-Christian Disease) of the mandible is presented. Multi-modal approach to management is discussed.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell/diagnosis; Histiocytosis, Langerhans-Cell/surgery*
  6. Azreen AB, Kwan CY, Prepagaren N
    Med J Malaysia, 2012 Dec;67(6):627-8.
    PMID: 23770962 MyJurnal
    Langerhan cell histiocytosis is a rare disease and usually occurs in paediatric age group. This disease may involve single or multiple organs system and has an unpredictable course of disease. The involvement of head and neck region are almost 90% of cases, however maxillary sinus involvement is very rare. We report a case of 2 year old boy presented with multi organ LCH (orbit, skull, sinus and liver). The mainstay treatment for this high risk multi organ LCH group is chemotherapy. Unfortunately, although with the advancement of treatment, their mortality rate is still high.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell*
  7. Yap FBB
    Dermatol. Online J., 2009;15(1):2.
    PMID: 19281707
    A 36-year-old Malaysian woman with erosive arthritis presented with multiple asymptomatic skin colored papulonodules on the hands, elbows, knees, ears, and feet; many macules, papules, and plaques were photodistributed. Progressive, erosive arthritis began about 18 months after the onset of skin involvement. Initially the patient was diagnosed to have dermatomyositis due to her skin findings, but later proved to have multicentric reticulohistiocytosis based on the typical histopathological features of histiocytic multinucleated giant cells with eosinophilic ground glass cytoplasm on skin biopsy. Workup for associated diseases including malignancies was negative. Her skin lesions and joint pain responded to treatment with methotrexate and prednisolone.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/pathology*
  8. Abdul Aziz DA, Abdul Rahman NA, Tang SF, Abdul Latif H, Zaki FM, Annuar ZM, et al.
    BMJ Case Rep, 2011;2011.
    PMID: 22674944 DOI: 10.1136/bcr.09.2011.4734
    Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/radiography; Histiocytosis, Langerhans-Cell/surgery*
  9. Shiran MS, Tan GC, Kenali MS, Sabariah AR, Pathmanathan R
    Malays J Pathol, 2008 Jun;30(1):63-5.
    PMID: 19108414 MyJurnal
    Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a systemic disease involving nodal and extranodal tissues. We report a 48-year-old female with recurrent nasal obstruction due to polypoidal masses involving the nasal sinuses, turbinates and septum bilaterally, and lumps in the right infra-orbital region and region of the right lacrimal sac. A 4 cm right upper neck mass was also noted, which was initially diagnosed as histiocytic lymphoma. Histopathology of the nasal and infraorbital lesions revealed fibro-inflammatory masses containing histiocytic cells with large vesicular nuclei and abundant foamy cytoplasm exhibiting emperipolesis and lymphophagocytosis, admixed with scattered plasma cells and lymphocytes. These histiocytes revealed immunohistochemical positivity for S-100 protein and CD68, but were negative for CDla. The findings supported a diagnosis of RDD. This report serves to remind pathologists and clinicians of the extranodal manifestations of RDD and its potential confusion with lymphomas.
    Matched MeSH terms: Histiocytosis, Sinus/complications; Histiocytosis, Sinus/diagnosis*; Histiocytosis, Sinus/metabolism
  10. Gan GG, Eow GI, Teh A, Ng SC, Sangkar JV
    Med J Malaysia, 2004 Mar;59(1):100-2.
    PMID: 15535343
    Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/diagnosis*; Histiocytosis, Non-Langerhans-Cell/drug therapy; Histiocytosis, Non-Langerhans-Cell/genetics*
  11. Oweity T, Scheithauer BW, Ching HS, Lei C, Wong KP
    J. Neurosurg., 2002 Feb;96(2):344-51.
    PMID: 11838810
    Erdheim-Chester disease (ECD) is a rare multiple system histiocytosis that is characterized pathologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The diagnosis is often confirmed by biopsy of bone or of orbital or retroperitoneal soft tissue. Intracranial involvement is rare. The authors report a case of ECD in which the diagnosis was made after biopsy of a hypothalamic mass. The mass had been discovered during a workup for panhypopituitarism in a 55-year-old man with urological and bone disease. Aside from diabetes insipidus, other features of pituitary insufficiency have seldom been reported and no patients have presented with a hypothalamic tumor. The endocrinological and neurological aspects of ECD are discussed, as is its differential diagnosis. Reported cases of the disorder associated with hypopituitarism or found during biopsy of central nervous system structures are also reviewed.
    Matched MeSH terms: Histiocytosis/complications*; Histiocytosis/pathology*; Histiocytosis/physiopathology
  12. Wong KK, Lin HP, Looi LM
    Int J Gynaecol Obstet, 1992 Oct;39(2):131-4.
    PMID: 1358712
    Vulvar ulceration is a rare manifestation of histiocytosis X. A 13-year-old girl had a nonhealing vulvar ulcer for 1 year. She had been in remission from histiocytosis X and the ulcer was not recognised as a sign of disease recurrence until tissue biopsy was obtained for histopathological and immunohistochemical studies. This article stresses the importance of establishing an accurate diagnosis when chronic vulvar ulcers are encountered and reviews the literature on this uncommon presentation of histiocytosis X.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell/complications*; Histiocytosis, Langerhans-Cell/diagnosis; Histiocytosis, Langerhans-Cell/pathology
  13. Ramanathan M, Duraisamy G
    Ann Acad Med Singap, 1991 Nov;20(6):803-4.
    PMID: 1803975
    Virus associated haemophagocytic syndrome (VAHS), a distinct clinico-pathologic entity, is characterised by systemic proliferation of non-neoplastic histotiocytes showing haemophagocytosis resulting in blood cytopaenia. It has been described in relation to several viruses earlier. Here we describe a young girl who developed this reactive process during the course of dengue haemorrhagic fever (DHF).
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/etiology*
  14. Mohd Fuad SK, Adznan N, Hayati AR, Virasamy V, Rahim NS
    Malays J Pathol, 2020 Dec;42(3):439-444.
    PMID: 33361726
    INTRODUCTION: Chronic histiocytic intervillositis (CHI) is a rare placental lesion strongly associated with recurrent miscarriages and fetal losses. It requires histopathological diagnosis and can only be made after delivery of the products of conception (POC). We describe a case of CHI in a 41-yearold lady with a 16-year history of thirteen recurrent consecutive first trimester miscarriages.

    CASE REPORT: The patient is a 41-year-old lady who suffered first trimester miscarriages in all her thirteen pregnancies. The relevant clinical investigations revealed neither significant nor helpful findings in determining the cause of recurrent miscarriages. Histological findings in each except one of the submitted conceptual tissue showed similar features of histiocytic aggregates primarily within the intervillous spaces, a characteristic description of CHI. One of the samples showed degenerative changes.

    DISCUSSION: Practicing pathologists are not familiar with the histological features of CHI and this may be a potential pitfall in routine examination of POCs. Recognising this entity allows for accurate diagnosis and hence better management. The aetiology remains unclear, although an immunopathological basis are being explored.

    Matched MeSH terms: Histiocytosis/pathology
  15. Low DE, Tang MM, Surana U, Lee JY, Pramano ZAD, Leong KF
    Int J Dermatol, 2019 Oct;58(10):e190-e193.
    PMID: 31192449 DOI: 10.1111/ijd.14518
    Matched MeSH terms: Histiocytosis/diagnosis*; Histiocytosis/genetics; Histiocytosis/pathology
  16. Ahmad AR, Ismail SM, Zain RB, Mohamad Zaini Z
    Ann Dent, 2009;16(1):31-36.
    MyJurnal
    Langerhans Cell Histiocytosis (LCH) refers to a group of lesions presenting with a spectrum of clinical. features but sharing similar histology. These lesions are rare and treatment has been quite variable with current treatment protocol recommended being dependent on whether it is a unifocal or multi focal bone disease or a multi focal multisystem disease. However, the clinical presentations of LCH are variable and the decision to place into the appropriate clinical types may sometimes be masked by the non-discovery of all the lesions. In the oral maxillofacial area, the clinical features of these lesions may further pose a problem by nondescript manifestations as dental/periodontal/oral mucosal disorders. These oral findings may sometimes lead to inappropriate choice of treatment and delay in the diagnosis of all the lesions involved. This paper describes one such case where LCH manifest itself as a periodontal disease thus leading to delay in identifying all the sites involved and consequently a delay in id~ntifying the appropriate clinical type.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell
  17. Ibrahim ZA, Lung WS, Long PK
    Malays J Med Sci, 2009 Jan;16(1):48-50.
    PMID: 22589649 MyJurnal
    Langerhans cell histiocytosis is a disease primarily affects the bone. More than 50 percent of the disease occurs between the age of 1 and 15. We reported a case of a 2 year old boy who presented with a gluteal mass. Radiographic imaging showed an osteolytic lesion suspicious of malignancy. However, the histological diagnosis was Langerhans cell histiocytosis.
    Matched MeSH terms: Histiocytosis, Langerhans-Cell
  18. Khoo JJ, Rahmat BO
    Malays J Pathol, 2007 Jun;29(1):49-52.
    PMID: 19105329 MyJurnal
    Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
    Matched MeSH terms: Histiocytosis, Sinus/metabolism; Histiocytosis, Sinus/pathology*
  19. Leong CF, Cheong SK, Hamidah NH, Ainoon O, Kannaheswary Y
    Malays J Pathol, 1998 Dec;20(2):103-8.
    PMID: 10879271
    A 40-day-old baby girl presented with intermittent fever, lymphadenopathy, massive hepatosplenomegaly, progressive pancytopenia and features of disseminated intravascular coagulopathy. A bone marrow aspiration was performed and showed florid histiocytic proliferation with marked hemophagocytosis. Based on the diagnostic guideline for Hemophagocytic Lymphohistiocytosis proposed by the Familial Hemophagocytic Lymphohistiocytosis Study Group of Histiocyte Society, this patient has fulfilled most of the criteria. We have also found that serum ferritin and lactate dehydrogenase to be very high in this patient. It remains uncertain whether the disorder is reactive or neoplastic.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/blood*; Histiocytosis, Non-Langerhans-Cell/pathology
  20. Ramanathan M, Karim N
    Med J Malaysia, 1993 Jun;48(2):240-3.
    PMID: 8350805
    This report deals with a young man who developed features of haemophogocytosis during the course of typhoid fever. The pertinent clinical and laboratory features of typhoid-associated haemophagocytosis are discussed. The need for blood component replacement therapy in addition to specific anti-microbials to treat haemophagocytosis complicating typhoid fever is stressed.
    Matched MeSH terms: Histiocytosis, Non-Langerhans-Cell/diagnosis; Histiocytosis, Non-Langerhans-Cell/etiology*
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