Histiocytosis x by dr kartik sood
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Histiocytosis X refers to a group of syndromes involving abnormal proliferation of histiocytes, a type of immune cell. Langerhans cell histiocytosis is a specific disease within this group characterized by abnormal proliferation of Langerhans cells in tissues. Pulmonary Langerhans cell histiocytosis primarily affects young adult smokers and presents with nonspecific symptoms like cough, breathlessness, and chest pain. Diagnosis involves chest imaging showing characteristic nodules and cysts as well as pathology identification of Langerhans cells. Treatment involves smoking cessation and corticosteroids or chemotherapy for severe disease.
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![WHAT IS HISTIOCYTOSIS X? Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes. ,[object Object],The X refers to the unknown entities of the disorder as there were and still are many questions about the etiology of this disorder](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)
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- LCH was first reported in 1893 and involves abnormal accumulation of dendritic cells called Langerhans cells in various organs.
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Se describe la leucemia cutis en cuanto a sus manifestaciones cutáneas y a las características diagnósticas observadas en la microscopía en dicha enfermedad.
Se debe sospechar esta enfermedad en pacientes con diátesis hemorrágica, y muchos otros signos y síntomas, además de la relación con otras enfermedades como procesos infecciosos / inmunocomprometidos, pioderma gangrenoso, eritema nodoso, paniculitis, eritema multiforme, urticaria, otras dermatosis refractarias al tratamiento convencional inicial.
Hay infiltración de células leucémicas, y también otras alteraciones no específicas. Esto llevará a cambiar el pronóstico del estado de salud del paciente.
Existe la variante aleucémica.
Es una presentación infrecuente en la que la piel está infiltrada por células leucémicas, en ausencia de leucemia demostrable en sangre periférica o en médula ósea.
Es imprescindible realizar estudio histológico de la piel e inmunofenotipificación.
Más información en:
www.patologica.com
Patologica.com
www.apatologica.blogspot.com
www.apatologica.blogspot.co.uk
www.apatologica.blogspot.com.es
Referencias:
Agrawal AK, Guo H, Golden C. Siblings presenting with progressive congenital aleukemic leukemia cutis. Pediatr Blood Cancer. 2011;57(2):338-40.
Birgit Federmann, Manuel Abele, David Santiago Rosero Cuesta, Wichard Vogel, Leonardo Boiocchi, Lothar Kanz, Leticia Quintanilla-Martinez, Attilio Orazi, Irina Bonzheim, Falko Fend.The detection of SRSF2 mutations in routinely processed bone marrow biopsies is useful in the diagnosis of chronic myelomonocytic leukaemia. Human Pathology 2014;45(12):2471–2479.
Iitani MM, Abe R, Yanagi T, et al. Aleukemic leukemia cutis with extensive bone involvement. J Am Acad Dermatol. 2010 Sep;63(3):539-41.
Martínez-Poventud G, Fradera J, Pérez S, et al. Aleukemic leukemia cutis preceding acute monocytic leukemia: a case report. PRHSJ. 2008;27(3):256-8.
Najem N, Zadeh VB, Badawi M, et al. Aleukemic leukemia cutis in a child preceding T-cell acute lymphoblastic leukemia. Pediatr Dermatol. 2011;28(5):535-7.
Yoder FW, Schuen RL. Aleukemic leukemia cutis. Arch Dermatol. 1976; 112: 367-9.
Yonal I, Hindilerden F, Coskun R, et al. Aleukemic Leukemia Cutis Manifesting with Disseminated Nodular Eruptions and a Plaque Preceding Acute Monocytic Leukemia: A Case Report. Case Rep Oncol. 2011;4:547–54.
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The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
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Hurler Syndrome is a rare genetic disorder caused by the absence of an enzyme that breaks down complex sugars, causing them to build up in the lysosome and produce severe symptoms. Sidney Van Hook, a three-year-old with Hurler Syndrome, received a bone marrow transplant which was successful but she still has some symptoms. New medications like Aldurazyme can replace the missing enzyme to stop sugar buildup and bone marrow transplants or gene therapy may help treat the disease.
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International Journal of Recent Trends in Science and Technology Volume 10, Issue 2, 2014.
http://statperson.com/Journal/ScienceAndTechnology/Volume10Issue2.php
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Gargoylism
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There is an excessive intracellular accumulation of both Chondroitin Sulfate B and Heparitin Sulfate in those tissues and organs where they are normally found.
Inherited as an autosomal recessive trait.
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Histiocytosis x by dr kartik sood
- 1. HISTIOCYTOSIS X DR KARTIK SOOD JUNIOR RESIDENT DEPTT OF PUL MED
- 3. INTRODUCTION AND BACKGROUND Sometimes referred to as histiocytosis x Lch is a disease of abnormal clonal proliferation of a unique type of cell in the monocyte-macrophage cell line known as the langerhans cell. It is named after the medical student paullangerhans, the first scientist to describe the cell (1868).
- 4. CLASSIFICATION OF THE HISTIOCYTOMAS
- 8. Skin: seborrheic eruptions on the trunk and scalp
- 9. Hepatosplenomegaly, lymphadenopathy, lung lesions and osteolytic bone lesions are present
- 10. Bone marrow = anemia/thrombocytopenia
- 11. 50% chance of 5 year survival
- 13. Birbeck Granules Plasma membrane invaginations Rodlike structures with a dilated end -discribed as “tennis raquets” Their presence on biopsy confirms the diagnosis
- 14. Etiology Much debate still exists as to whether this disease is immunologically based, viral or neoplastic Due to the clonality of CD1a+ histiocytes, current opinion holds that it is a clonal neoplastic disease with variety of presentations that differ in severity and behavior
- 15. PULMONARY LANGERHANS’-CELL HISTIOCYTOSIS Pulmonary langerhans’-cell histiocytosis, eosinophilicgranuloma of the lung is characterized by abnormal organ infiltration by langerhans’ cells. Smoking-related, interstitial lung disease that primarily affects young adults (20 to 40 years of age) Pulmonary Langerhans’-cell histiocytosis is not a granulomatous disorder. Moreover, the lesions are often devoid of eosinophils. Thus, the older term,eosinophilicgranuloma, is a misnomer.
- 16. PATHOGENESIS The pathogenesis of pulmonary Langerhans’-cell histiocytosis is unknown One hypothesis of disease pathogenesis, the bombesin hypothesis, contends that increased bombesin like peptide production plays a central role . Bombesin is a neuropeptide produced by neuroendocrine cells, which are increased in the lungs of smokers.
- 17. bombesin-like peptides (BLP) tobacco glycoprotein (TGP) granulocyte-macrophage colony-stimulating factor [GM-CSF])
- 25. Hemoptysis is occasionally reported, and it should prompt consideration of superimposed infection ( Aspergillus)
- 27. INVESTIGATIONS CHEST RADIOGRAPH - very characteristic if not diagnostic. The combination of ill-defined or stellate nodules (2 to 10mm in size), reticular opacities, upper-zone cysts or honeycombing,preservation of lung volume, and costophrenic angle sparing are believed to be highly specific for this disorder.
- 29. COMPUTED TOMOGRAPHY Combination of multiple cysts and nodules with a middle to upper-zone predominance with interstitial thickening in a young smoker is characteristic and diagnostic of PLCH .Honeycombing can be seen in advanced disease.
- 31. Magnetic Resonance Imaging The role of magnetic resonance imaging in pulmonary Langerhans’-cell histiocytosis is limited to the evaluation of bony and central nervous system lesions.
- 32. BAL FLUID ANALYSIS Bronchoalveolarlavage (BAL) can be of diagnostic value in cases of suspected histiocytosis X. the CD4:CD8 ratio may be decreased. Langerhans’ cells in BAL can be recognized by their characteristic staining for S-100 protein or peanut agglutination antigen. These cells are also OKT-6 (CD-1) positive, are identified by a specific monoclonal antibody (MT-1), and contain characteristic Birbeck bodies on electron microscopic evaluation
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