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May 2016, Moscow, Russia
OF PEDIATRIC HEMATOLOGY and ONCOLOGY
HISTIOCYTOSIS
ABSTRACT NO.: P-166
Clinical case of verification of the Langerhans cells histiocytosis diagnosis
Yu.V. Shatohin1, E.D. Teplyakova1, I.V. Snezhko1, E.I. Kuzub1, O. Shatokhina2
1Rostov State Medical University, Rostov-on-Don, Russia; 2Rostov Cancer Research Institute, Rostov-on-Don, Russia
Key words: clinical case, histiocytosis
Introduction. Histiocytosis are a heterogeneous group of diseases, whose pathologic substrate are the cells of histiocytic series which include: tissue macrophages, monocytes, dendritic cells and their precursors.
Aim. Langerhans cells histiocytosis is a disease characterized by proliferation of cells of the macrophage phagocyte system in the skin, bones, bone marrow, liver, spleen, lungs, lymph nodes and in other organs and tissues. How a variety of clinical symptoms in Langerhans cell histiocytosis. How a variety of clinical symptoms in Langerhans cell histiocytosis with this pathology show clinical case.
Materials and methods. Patient N., 30 y.o., contacted the clinic with complaints of weakness, malaise, rashes on the scalp, face, armpits, groin and perianal area. He has been ill since the spring of 2008 when he first noticed scalp rash. The patient consulted a dermatologist in 4 months, his condition was considered as seborrheic dermatitis manifestation and local treatment was prescribed. The therapy gave no results and in a month the rash spread to the skin of the back, armpits and groin; scrapings detected the spores of Mucor fungi. Antifungal therapy was conducted with little positive effect. In 2009 the concilium in Skin and Venereal Diseases Dispensary regarded the patient's condition as chronic benign Hailey-Hailey pemphigus, and the patient had a course of treatment with a positive effect in the form of reduced hyperemia and skin elements moisture. However, after the cancellation of prednisolone skin lesions began to progress again. In 2012 the patient was diagnosed diabetes insipidus and autoimmune thyroiditis with hypothyrosis and the patient regularly took Minirin and Eutiroks. In 2013 the patient developed spontaneous pneumothorax. In March 2014 computer tomography revealed pneumosclerosis with bullous emphysema from centrilobular to panlobular all over the lungs. Mediastinal lymphatic nodes were not enlarged.
By 2014 papular, pyodermic , seborrheic skin lesions were recorded on the chin. The biopsy of skin lesions was performed in September 2015. Histological and immunohistochemical study revealed: CD68 - positive granular in some cells; S100 - positive in a significant number of cells; Langerin - positive in a large proportion of cells; CD20 - positive in clusters of the few B cells; CD3 - positive in the few T-cells; Ki67 - negative 2 times, which allowed to establish the diagnosis of Langerhans cell histiocytosis.
Results. At the time of his visit to the clinic the patient had the following manifestations of the disease: polymorphic skin rash (local seborrheic, infiltrative-erosive, papular, ^
pyodermic) on the face, scalp and in skin folds (axillary, inguinal and perianal area); diabetes insipidus; hypothyrosis; hepatomegaly; multiple cystic lesions of lung tissue. uo
Conclusion. The above clinical case report shows little awareness of physicians of histiocytosis, which led to the late verification of the diagnosis (the diagnosis was established
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7 years later the clinical manifestation of the disease). Such late diagnostics undoubtedly led to the progression of the disease and multiple visceral lesions most of which
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AoSTcACT disease oriented
SOCltTt INTERNATIONALE D'ONCOLOGIE PEHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: OP-183
Late diagnosis of Langerhans cell histiocytosis (LCH) in children
D. Evseev, I.I. Kalinina, T.Yu. Salimova, D.D. Baydildina, А.А. Maschan, М.А. Maschan
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia
Key words: Langerhans cell histiocytosis, difficulties of diagnosis, hepatic fibrosis, damage of intestines
Introduction. Langerhans cell histiocytosis (LCH) in children is extremely rare. The main clinical manifestations are rash, bone involvement , rare - the involvement of liver, spleen, bone marrow, lungs.
Aim. To describe clinical cases of the late diagnosis of LCH due to rare clinical manifestations (damage of intestines, hepatic fibrosis). Materials and methods. There were examined 2 clinical cases in which there were difficulties in diagnosis of LCH. Results. We present the description of the cases.
Patient № 1. A girl, the onset at the age of 4 months with the appearance of rash, a biopsy was not performed, rash reduced spontaneously. At the age of 6 months - fever, blood in stool, a colonoscopy with a biospy were performed. Diagnosis: total erosive colitis. During histological investigation LCH (medium-sized cells with high nucleocytoplasmic ratio, oval hyperchromic nuclei ("coffee bean") positive for CDIa and S100, CD68,) was detected. At the moment of the transferral to the FRC PHOI - rash, hepatosplenomegaly, anaemia, thrombocytopenia. The child received therapy according to the LCH III protocol with a positive effect.
Patient № 2. A boy, the onset at the age of 2 years, started with fever, hepatomegaly (ultrasound investigation showed inhomogeneous cystic ectasia of segmental bile ducts, a choledochous duct), the diagnosis of Caroli disease was made. The Kasai operation was performed: laparotomy, cholecystectomy, excision of the choledochous duct, applying porto-jejunal anastomosis (a biopsy was not performed) without changes over time. During investigation before the liver transplantation the computed tomography of the lungs showed multiple air-filled cystic cavities, in the form of fibrocystic transformation of the lung tissue. In order to verify the diagnosis the puncture biopsy of the liver was performed. According to the histological investigation: periportal infiltration with lymphocytes, with the presence of single eosinophilic leukocytes and a small accumulation of histiocytes around one tract, positive for CDIa and Langerin. 2 courses of therapy (IC 1 and IC 2) were performed- without effect. At the moment the child is alive, the involvement of the lungs and the liver are considered to be permanent LCH complications, symptomatic treatment is being carried out.
Conclusion. Due to the rare occurrence of LCH in children and difficulties of its diagnosis without specific clinical picture the diagnosis is made late that may result in the unfavourable outcome.
ABSTRACT NO.: OP-313
Histiocytic inflammatory response as predictor of relapse in hematological and lymphoid malignancy
Atish Narayanrao Bakane, Revathi R Raj, Ramya S Uppuluri, Divya S Subburaj, Sreejith R Kurup
Apollo Speciality Hospital, India
Key words: histiocytic inflammatory response, retrospective observational study, xanthogranulomatous histiocytosis
Introduction. There have been a few case reports published in literature documenting histiocytic inflammatory response in children with hematological and lymphoid malignancies.
We present a series of 2 cases wherein histiocytosis was found to precede relapse of leukemia and lymphoma.
Aim. To substantiate that histiocytic inflammatory response may be a predictor of relapse of leukemia and lymphoma.
Materials and methods. This is a retrospective observational study of 2 children with hemato-lymphoid malignancies treated at Apollo Speciality Hospital, Chennai which is a tertiary referral centre in India.
The first child is an 8 year old male child who was diagnosed to have CALLA positive ALL in 2014 and received chemotherapy as per UKMRC ALL 2011 protocol. He achieved complete remission at the end of induction and was continued on consolidation, delayed intensification and maintenance chemotherapy. During cycle 3 of maintenance chemotherapy, he was noted to have painful swelling of the right knee joint. Imaging revealed osteolytic lesion involving the lower end of femur. Biopsy with histopathology and immunohistochemistry confirmed Langerhans cell histiocytosis. He was accordingly started on Vinblastine and prednisolone. In view of persistent disease, he then received 2 cycles of cladribine and ¡¿2 cytarabine. After 2 cycles, PET CT revealed persistent lesion in the right femur. In view of fever, bone marrow was repeated which confirmed isolated bone marrow relapse of ALL.
q The second child is a 16 year old male who was diagnosed to have Stage 2 Hodgkin lymphoma at Bangladesh in 2008 and had received 6 cycles of ABVD before being referred to our
centre. Assessment showed persistent disease in few mediastinal lymph nodes. He was given involved field radiotherapy and was on follow up. In 2013, in view of abdomen pain U and USG revealing multiple enlarged lymph nodes, laparoscopic excision biopsy was done. Histopathology confirmed xanthogranulomatous histiocytosis. He received Celecoxib
and oral steroids after which he was in remission. In 2016, PET CT was repeated in view of fever and loss of appetite, which revealed multiple enlarged lymph nodes with significant ¡>2 FDG uptake. Biopsy, histopathology and IHC confirmed T cell rich B cell lymphoma.
1 Results. The first child was given reinduction chemotherapy followed by MEC in view of persistent disease. He remained refractory with progressive disease and ultimately succumbed
to his illness. The second child has been started on salvage chemotherapy at the time of submission of this abstract. We plan to consolidate with autograft once in remission Conclusion. Children with hematological and lymphoid malignancies who develop histiocytic inflammation during chemotherapy or when in remission, should be followed up with a high index of suspicion for relapse. Early diagnosis of relapsed disease and institution of appropriate treatment for both histiocytosis and malignancy may improve outcomes in these children.
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May 2016, Moscow, Russia
OF PEDIATRIC HEMATOLOGY and ONCOLOGY
ABSTRACT NO.: OP-314
Incidence and clinical spectrum of drug resistant bacterial infections and associated mortality in pediatric hemato-oncology: experience from a tertiary care centre in South India
Atish Narayanrao Bakane, Revathi R Raj, Sreejith R Kurup, Ramya S Uppuluri, Divya S Subburaj
Apollo Speciality Hospital, India
Key words: drug resistant bacterial infections, hemato-oncological conditions, resistance to carbapenems
Introduction. There has been a well documented upsurge in the prevalence of drug resistant bacterial infections among children with hemato-oncological conditions undergoing treatment. Knowledge with regards to the above can pave the way to better antibiotic stewardship and improved preventive strategies to minimize irrational use. We present data on the incidence and clinical spectrum of bacterial infections among this group of children, patterns of resistance and mortality associated. Aim. To describe Incidence and clinical spectrum of drug resistant bacterial infections and associated mortality in pediatric hemato-oncology.
Materials and methods. We conducted a retrospective, observational study from January 2014 to December 2015, with regards to the clinical spectrum, incidence and prevalence of bacterial infections among children undergoing treatment for hemato-oncological conditions and haematopoeitic stem cell transplants at a tertiary care centre in South India. We analyzed the incidence of multidrug resistant sepsis, defined as resistance to carbapenems and studied the correlation between multidrug resistant sepsis and morbidity and mortality. Various interventions have been instituted in order to reduce sepsis and improve outcomes after discussion with the Hospital Infection Control (HIC) team. Results. The total inpatients at our centre in the last 2 years in the department of pediatric hemato-oncology from January 2014 to December 2015 have been 1252. The total number of culture positive sepsis in 2014 and 2015 amounts to 50. Among the children developing culture positive bacteremia, 11 were during AML induction chemotherapy, 14 were during ALL chemotherapy, 23 Post HSCT with GVHD, 1 during OEPA chemotherapy for Hodgkin lymphoma and 1 during chemotherapy for metastatic Ewing sarcoma. The organisms isolated included Gram negative bacilli including E coli, Klebsiella, Pseudomonas in 24, CONS in 15 (which were found to be insignificant), Enterobacter 3, Alpha hemolytic streptococci 2, Acinetobacter 3, Ralstonia 1, St. aureus 1, C. elegans 1.
Out of the 50 culture positive cases, carbapenem resistance was found in 15 cases (30 %). 2 cases were ESBL producers and sensitive to carbapenems. Mortality rate among children with multidrug resistant bacteremia is 2/15 (13 %).
The above data analysis led to several discussions with the Department of Hospital Infection Control. Interventions initiated in early 2015 included studying CARBA-R PCR in all patients admitted for HSCT and high dose chemotherapy. Positive CARBA R resulted in early initiation of Colistin. In children with Carbapenem resistant bacteremia, Colistin was added early. Preventive strategies included use of neutropenic care, specific neutropenic diet, use of granulocytes early prior to end organ damage.
Conclusion. Although the total number of children with culture positive bacteremia has been limited to 50 in the last 2 years at our centre, 30 % of these have been organisms with multidrug resistance, particularly to carbapenems. The most common organisms isolated are gram negative bacilli. Mortality due to drug resistant bacteria sepsis has been 13 % at our centre. Analysis of this data with regards to prevalent institutional flora and drug resistance has resulted in institution of several preventive strategies. These include increased care during neutropenic period and early institution of Colistin to reduce mortality and improve survival.
ABSTRACT NO.: O-343
Study of late effects and permanent consequences of pediatric onset Langerhans cell histiocytosis
G.O. Bronin, R.B. Miroshkin, E.A. Bezunov, E.V. Mironova, E.V. Fisun, Yu.V. Pashko, B.M. Kurmanov, E.A. Promyslova, L.V. Sidorenko, A.F. Karelin, V.N. Kasatkin, N.N. Volodin
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Moscow, Russia
Key words: l angerhans cell histiocytosis, permanent consequences, late effects, cognitive functions
Introduction. Langerhans cell histiocytosis (LCH) is a rare disease characterized by variable biological behavior and course ranging from rapid life-threatening to self-resolving forms. Permanent consequences (PC) can complicate both multi-system and single system LCH. Pathogenesis of PC remains obscure in many cases. Aim. The main objective was to evaluate clinical features and severity of permanent consequences among survivors of pediatric onset LCH.
Materials and methods. 25 LCH survivors of LCH aged from 5 to 17 were admitted to our Rehabilitation Center for special examination and rehabilitation program. The median of surveillance from the time of onset was 8.5 years. We performed standard clinical check-up with main focus of axiology and clinical features of LCH and its PC. Large number of biochemistry and endocrinology tests was done and neurological survey according to the Extended Disability Status Scale (EDSS) and International Cooperative Ataxia Rating Scale u
(ICARS) was performed as well. We used a battery of Neuropsychology tests of CANTAB eclipse (Cambridge Cognition, UK) for the assessment of cognitive functions. Q
Results. In 10 cases different PC were found. Orthopedic problems were revealed in 4 cases and demanded physical treatment. Neurodegenerative disease was proven both uo
by magnet resonance imaging and by neurological assessment in two boys. The intravenous immunoglobulin treatment was initiated in both cases. Correction of hormone substitution ^
therapy demanded in 5 patients. The main cognitive functions remained intact in most cases. We proved a good level of planning skills, mastering the strategy, spatial memory, often ahead of age. Nevertheless, the results of this study allowed us to determine areas of concern in the cognitive functions of the respondents, in particular, a tendency to impulsiveness and lack of working memory capacity. Both disorders were associated with the frontal cortex location.
Conclusion. The PC in survivors of LCH can be found with relatively high frequency and can affect their quality of life. Special examination and sometimes treatment is needed even in long term follow-up time after the discontinuation of the main therapy.
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AoSTcACT disease oriented
SOCIÉTÉ INTERNATIONALE D'ONCOLOGIE PÉHATRIQUE
SIOP ASIA CONGRESS
ABSTRACT NO.: PP-360
Langerhans cell histiocytosis (LCH) of the female genital tract: A case report and review of the literature
Husam Farid Bashir, Tal A Dujovny, Hertzel Gabriel, Yoseph Horovitz
HaEmek Medical Center, Afula, Israel
Key words: Langerhans cell histiocytosis, vulvar, LCH
Introduction. Langerhans cell histiocytosis (LCH) of the female genital tract is rare, LCH involving the genital tract, including the vagina, cervix, uterus, ovaries, and vulva has been reported in adults. Primary LCH of the vulva is rare and even rarer in children. Because of the small number of cases reported, there is no standard recommendation for the management of genital LCH. Aim. Case report and review of literature.
Materials and methods. Methods of treatment include topical steroids, oral steroids, topical nitrogen mustard, thalidomide, interferon, surgery (vulvectomy or local excision), radiotherapy and chemotherapy (vincristine, vinblastine, methotrexate, 2-chlorodeoxyadenosine).
Results. We report a case of a 14 year old child with vulvar LCH. After initial evaluation, she was treated according to the LCH-IV protocol, STRATUM I, with vinblastine + prednisone. We describe the clinical presentation, evaluation, and treatment of primary vulvar LCH along with review of the literature.
ABSTRACT NO.: P-380
Histiocitosis of cells de Langerhans. Series of cases in pediatric population. National institute of Oncology and Radiobiology (INOR) 1980-2015
Migdalia Perez, Mariuska Forteza, Jose Alert, Jesus Reno
National institute of Oncology and Radiobiology of Cuba, Havana
Key words: Langerhans cell histiocytosis
Introduction. Langerhans cell histiocytosis is a rare disease with different clinical and pathological presentation, is clinically divided into three groups: unifocal, multifocal unisystem, and multifocal multisystem. This enigmatic entity's etiopatogenia is not all right clarified. As of the present moment, investigators's bigger number presents that one is produced. Alteration in the regulation of the immune system in these patients. The presence of aggregates of another immunogenicly active cells in injuries, the anomalies found in the swindle, the decrease in linfocitos's number T suppressing CD8+ and the increase in several citocinas's synthesis (factor macrofagos stimulator and little granules, IL-1, IL-3, IL-8, IL-10, TNF a and interferon) suggest a physiological answer exaggerated of the CL to one Identified antigen or, on the contrary, an appropriate answer to abnormal appropriate signals of another cells of the immune system.
Aim. To characterize the pediatric patients with diagnostic of Langerhans cell histiocytosis tried in the National Institute of Oncology (INOR) during the period 1980-2015. Materials and methods. A descriptive and retrospective, pediatric-patients enrolled in the Institute Nacional of Oncología and Radiobiología (INOR) with diagnosis of Histiocitosis of cells of Langerhans during the period understood among the year 1980 and the 2008 study came true. Our sign of study got constituted for 32 patients with equal age or minor to 15 years registered in the INOR with diagnosis of Histiocitosis of cells of Langerhans and that they received the initial treatment in the INOR during the mentioned period of time. The bibliographic revision of the theme in point came true. The data got from case histories themselves, the archives of morbid anatomy were consulted and correspondent data bases of oncopediatria's service of the INOR and of the Record Nacional of Cancer.
He manufactured a model for the anthology of primary data, created for the purpose (I Annex # 1); Where they included the related variables to: The patient's general data, clinical, diagnosis, treatment elements and tracking.
The secondary data obtained of the filling of the model got into a data base using the program ACCESS 2003 and they processed themselves utilizing the statistical system SPSS 11,5 for WINDOWS.
Results. The masculine sex prevailed (65.6 %) with ages of smaller or similar to 2 years (40.6 %). the located bony pain Prevailed (84.4 %), the bony affectation (90.6 %)
and the located illness (84.4 %). the stratum Prevailed of diagnostic presumptive (65.6 %). The used treatment was the chemotherapy like unique and combined modality;
¡^ with the protocol of sequential oral chemotherapy. The survival free of progression and global survival to the 10 years of 73.1 % and 86.9 %, respectively.
O Conclusion. Langerhans cell histiocytosis is an illness of systemic commitment, with diverse forms of clinical presentation, what makes it an entity of difficult diagnostic
>- and handling. In the analysis of survival, association was observed statistically significant with regard to the age, the extension of the illness and the answer to the treatment.
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