Publications by authors named "Vasanta Nanduri"

17 Publications

  • Page 1 of 1

Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study.

Blood Adv 2020 08;4(15):3754-3766

Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden.

We report the largest prospective study thus far on hematopoietic stem cell transplantation (HSCT) in hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. Although all patients with HLH typically need intensive anti-inflammatory therapy, patients with FHL also need HSCT to be cured. In the international HLH-2004 study, 187 children aged <18 years fulfilling the study inclusion criteria (5 of 8 diagnostic criteria, affected sibling, or molecular diagnosis in FHL-causative genes) underwent 209 transplants (2004-2012), defined as indicated in patients with familial/genetic, relapsing, or severe/persistent disease. Five-year overall survival (OS) post-HSCT was 66% (95% confidence interval [CI], 59-72); event-free survival (EFS) was 60% (95% CI, 52-67). Five-year OS was 81% (95% CI, 65-90) for children with a complete response and 59% (95% CI, 48-69) for those with a partial response (hazard ratio [HR], 2.12; 95% CI, 1.06-4.27; P = .035). For children with verified FHL (family history/genetically verified, n = 134), 5-year OS was 71% (95% CI, 62-78) and EFS was 62% (95% CI, 54-70); 5-year OS for children without verified FHL (n = 53) was significantly lower (52%; 95% CI, 38-65) (P = .040; HR, 1.69; 95% CI, 1.03-2.77); they were also significantly older. Notably, 20 (38%) of 53 patients without verified FHL had natural killer cell activity reported as normal at diagnosis, after 2 months, or at HSCT, suggestive of secondary HLH; and in addition 14 (26%) of these 53 children had no evidence of biallelic mutations despite having 3 or 4 FHL genes analyzed (natural killer cell activity not analyzed after 2 months or at HSCT). We conclude that post-HSCT survival in FHL remains suboptimal, and that the FHL diagnosis should be carefully investigated before HSCT. Pretransplant complete remission is beneficial but not mandatory to achieve post-HSCT survival. This trial was registered at www.clinicaltrials.gov as #NCT00426101.
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http://dx.doi.org/10.1182/bloodadvances.2020002101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7422132PMC
August 2020

Management of severe pulmonary Langerhans cell histiocytosis in children.

Pediatr Pulmonol 2020 08 8;55(8):2074-2081. Epub 2020 Jun 8.

Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Houston, Texas.

Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.
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http://dx.doi.org/10.1002/ppul.24822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7771630PMC
August 2020

Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study.

Blood 2017 12 21;130(25):2728-2738. Epub 2017 Sep 21.

Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the HLH-94 study, with an estimated 5-year probability of survival (pSu) of 54% (95% confidence interval, 48%-60%), systemic therapy included etoposide, dexamethasone, and, from week 9, cyclosporine A (CSA). Hematopoietic stem cell transplantation (HSCT) was indicated in patients with familial/genetic, relapsing, or severe/persistent disease. In HLH-2004, CSA was instead administered upfront, aiming to reduce pre-HSCT mortality and morbidity. From 2004 to 2011, 369 children aged <18 years fulfilled HLH-2004 inclusion criteria (5 of 8 diagnostic criteria, affected siblings, and/or molecular diagnosis in FHL-causative genes). At median follow-up of 5.2 years, 230 of 369 patients (62%) were alive (5-year pSu, 61%; 56%-67%). Five-year pSu in children with (n = 168) and without (n = 201) family history/genetically verified FHL was 59% (52%-67%) and 64% (57%-71%), respectively (familial occurrence [n = 47], 58% [45%-75%]). Comparing with historical data (HLH-94), using HLH-94 inclusion criteria, pre-HSCT mortality was nonsignificantly reduced from 27% to 19% ( = .064 adjusted for age and sex). Time from start of therapy to HSCT was shorter compared with HLH-94 (020 adjusted for age and sex) and reported neurological alterations at HSCT were 22% in HLH-94 and 17% in HLH-2004 (using HLH-94 inclusion criteria). Five-year pSu post-HSCT overall was 66% (verified FHL, 70% [63%-78%]). Additional analyses provided specific suggestions on potential pre-HSCT treatment improvements. HLH-2004 confirms that a majority of patients may be rescued by the etoposide/dexamethasone combination but intensification with CSA upfront, adding corticosteroids to intrathecal therapy, and reduced time to HSCT did not improve outcome significantly.
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http://dx.doi.org/10.1182/blood-2017-06-788349DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5785801PMC
December 2017

Is it micropenis? Does size matter?

Arch Dis Child Fetal Neonatal Ed 2017 Jul 5;102(4):F345. Epub 2016 Dec 5.

Watford General Hospital, Watford, UK.

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http://dx.doi.org/10.1136/archdischild-2016-311874DOI Listing
July 2017

Presentation of childhood cancers to a paediatric shared care unit.

Arch Dis Child 2015 Dec 21;100(12):1131-5. Epub 2015 Aug 21.

Department of Paediatrics, Watford General Hospital, Watford, UK.

Objective: To describe the pathways by which children with cancer present to a shared care oncology unit.

Design: A population-based retrospective cohort study of children diagnosed with cancer between the years 2004 and 2014.

Setting: District General Hospital with a level 2 Paediatric Oncology Shared Care Unit.

Patients: 93 children aged 0-15 years .

Outcome Measures: Time to presentation (TTP) was defined as time from initial symptoms to time seen by secondary paediatrics. Time to diagnosis (TTD) was defined as time from initial symptoms to diagnosis at a Principal Treatment Centre. Patient pathways to diagnosis were mapped and routes for different cancers were compared.

Results: Only 2/93 cases (2.1%) in 10 years were referred via the 2-week pathway. Most presentations were acute via immediate general practitioner (GP) referral or self-referral to the emergency department 62/93 (67%). Leukaemia presented acutely and via the GP more often than via self-presentation to the emergency department 21/28 (75% vs 25%), while solid tumours were self-referred to the emergency department 21/34 (62% vs 38%) more often than via the GP. TTP and TTD were calculated for 87 patients. Wilms' tumour demonstrated the shortest median TTP of 7 days and TTD of 16 days. Lymphoma had the longest TTD, with TTP 107 days and TTD 120 days. Pathways to diagnosis via other specialties were longer.

Conclusion: The majority of children diagnosed with cancer present via acute services, with the route varying between tumour types. Only two cases in 10 years were referred via the 2-week pathway, thus challenging its relevance in the paediatric population.
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http://dx.doi.org/10.1136/archdischild-2015-308765DOI Listing
December 2015

Haematopoietic stem cell transplantation for refractory Langerhans cell histiocytosis: outcome by intensity of conditioning.

Br J Haematol 2015 Jun 27;169(5):711-8. Epub 2015 Mar 27.

CIBMTR® (Center for International Blood and Marrow Transplant Research), Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, USA.

Patients with Langerhans cell histiocytosis (LCH) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high-risk patients: the first involves the combination of 2-chlorodeoxyadenosine and cytarabine; the other approach is allogeneic haematopoietic stem cell transplantation (HSCT). Here we evaluated 87 patients with high-risk LCH who were transplanted between 1990 and 2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning (MAC): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant-related mortality (TRM). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning (RIC): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3-year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, P = 0·02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain.
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http://dx.doi.org/10.1111/bjh.13347DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4433436PMC
June 2015

Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years.

Pediatr Blood Cancer 2013 Feb 25;60(2):175-84. Epub 2012 Oct 25.

Department of Hematology and Oncology, Epidemiology and Biostatistics Section, Istituto G. Gaslini, Genova, Italy.

These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented.
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http://dx.doi.org/10.1002/pbc.24367DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4557042PMC
February 2013

Role of the 2-week urgent referral pathway in childhood cancer.

Arch Dis Child 2012 Mar 13;97(3):233-5. Epub 2012 Jan 13.

Department of Paediatrics, Watford General Hospital, Vicarage Road, Watford, Hertfordshire WD18 0HB, UK.

Objective: To evaluate the role of the urgent 2-week referral pathway in facilitating the early diagnosis of childhood cancer.

Design: Retrospective case notes review of all children referred via the pathway and all children diagnosed as having cancer via other means over a period of 3½ years (January 2007-July 2010).

Setting: District general hospital with a shared care oncology service.

Patients: 82 children aged 0-15 years.

Outcome Measures: Outcomes for children referred via the 2-week urgent referral pathway, including diagnosis, need for further investigations and need for further specialist input.

Results: 35 children were referred via the 2-week urgent referral pathway with suspected cancer. Only one of these children had a malignancy. 49% of children required no further investigations and 43% were discharged after the first appointment with no follow-up thought necessary. Over the same time period, 47 children were diagnosed as having cancer via other means.

Conclusion: The pickup rate of cancer among children referred via the 2-week urgent referral pathway is extremely low and the vast majority of cancer is diagnosed via different routes.
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http://dx.doi.org/10.1136/archdischild-2011-300783DOI Listing
March 2012

Superior vena cava obstruction: dangers of a missed diagnosis.

J Paediatr Child Health 2011 Mar;47(3):150-1

Watford General Hospital, Watford, UK.

We report the case of a 7 year old girl who presented to the Children's Emergency Department with a 6 week history of bilateral facial and neck swelling. She had felt generally unwell, tired, with a recent onset of dry cough, and had presented multiple times to her general practitioner (GP) who after initially unsuccessfully trying an antihistamine, had given her five courses of soluble Betamethasone (corticosteroid) over six weeks, for presumed allergy; this temporarily relieved her symptoms for a couple of days each time. On subsequent referral to Accident and Emergency she was found to have a superior mediastinal mass, with a left pleural effusion and mediastinal deviation to the right. Further investigation confirmed the diagnosis of a T-cell lymphoma causing superior vena cava obstruction of blood flow through the SVC to the right atrium and is a classical oncological emergency. This case report highlights the importance of recognizing superior vena cava obstruction and the need for awareness of malignancy as a differential diagnosis when initially presented with a child with non-specific respiratory findings. We highlight that acute tumour lysis syndrome, a life threatening metabolic emergency that results from massive cytolysis of malignant cells, may occur after a single dose of corticosteroids and one should be aware of this potentially life-threatening complication.
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http://dx.doi.org/10.1111/j.1440-1754.2010.01741.xDOI Listing
March 2011

Central nervous system disease in Langerhans cell histiocytosis.

J Pediatr 2010 Jun;156(6):873-881.e1

Department of Neuroradiology; Medical University of Vienna, Vienna, Austria.

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http://dx.doi.org/10.1016/j.jpeds.2010.03.001DOI Listing
June 2010

Paediatric presentation of familial cerebral cavernoma.

Pediatr Blood Cancer 2010 Mar;54(3):468-9

Watford General Hospital, Watford, UK.

We report the case of a 13-year-old male who presented with headaches and was presumed to have a brain tumour. He was subsequently found to have multiple cerebral cavernomas with haemorrhage and positive family history. We review the literature on familial cavernomas. Cerebral cavernous malformations (CCMs) are characterized by abnormally enlarged capillary cavities without intervening brain parenchyma [Verlaan et al. Neurology 2005; 65:1982-1983] that may involve any part of the central nervous system. Focal neurologic deficit and haemorrhage occur in 45% of children, higher than in adults [Stoeter. Neurosurg Rev 2001; 24]. Paediatric patients with symptomatic cavernous malformations should be treated surgically because of the risk of haemorrhage [Lee et al. Child's Nervous Syst 2008; 24:321-327].
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http://dx.doi.org/10.1002/pbc.22288DOI Listing
March 2010

High incidence of hearing loss in long-term survivors of multisystem Langerhans cell histiocytosis.

Pediatr Blood Cancer 2010 Mar;54(3):449-53

Department of Paediatrics, Watford General Hospital, Watford, UK.

Background: Ear involvement in the acute phase of Langerhans cell histiocytosis (LCH) is commonly seen and well documented, but the long-term sequelae are less well described, particularly in relation to hearing loss.

Methods: We investigated 40 patients with biopsy-proven multisystem LCH >5 years from the end of treatment, using detailed audiological assessment and CT/MRI imaging of the petrous temporal bones.

Results: The incidence of ear involvement in the acute phase of disease was 70%. Fifteen of the 39 patients tested (38%) had residual permanent hearing loss at long-term follow-up.

Conclusions: The incidence of hearing loss is much higher than has previously been reported in LCH, and may reflect a referral bias of young (<2 years) and more complex patients to our tertiary centre. However, the hearing loss appears to be highly specific to this patient group when compared to other long-term survivors of childhood cancers, probably due to the propensity of LCH to involve the ears. We therefore recommend audiology testing as an important part of long-term follow-up for patients with multisystem LCH.
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http://dx.doi.org/10.1002/pbc.22186DOI Listing
March 2010

Diagnostic omission errors in acute paediatric practice: impact of a reminder system on decision-making.

BMC Med Inform Decis Mak 2006 Nov 6;6:37. Epub 2006 Nov 6.

Children's Acute Transport Service (CATS), 44B Bedford Row, London, WC1H 4LL, UK.

Background: Diagnostic error is a significant problem in specialities characterised by diagnostic uncertainty such as primary care, emergency medicine and paediatrics. Despite wide-spread availability, computerised aids have not been shown to significantly improve diagnostic decision-making in a real world environment, mainly due to the need for prolonged system consultation. In this study performed in the clinical environment, we used a Web-based diagnostic reminder system that provided rapid advice with free text data entry to examine its impact on clinicians' decisions in an acute paediatric setting during assessments characterised by diagnostic uncertainty.

Methods: Junior doctors working over a 5-month period at four paediatric ambulatory units consulted the Web-based diagnostic aid when they felt the need for diagnostic assistance. Subjects recorded their clinical decisions for patients (differential diagnosis, test-ordering and treatment) before and after system consultation. An expert panel of four paediatric consultants independently suggested clinically significant decisions indicating an appropriate and 'safe' assessment. The primary outcome measure was change in the proportion of 'unsafe' workups by subjects during patient assessment. A more sensitive evaluation of impact was performed using specific validated quality scores. Adverse effects of consultation on decision-making, as well as the additional time spent on system use were examined.

Results: Subjects attempted to access the diagnostic aid on 595 occasions during the study period (8.6% of all medical assessments); subjects examined diagnostic advice only in 177 episodes (30%). Senior House Officers at hospitals with greater number of available computer workstations in the clinical area were most likely to consult the system, especially out of working hours. Diagnostic workups construed as 'unsafe' occurred in 47/104 cases (45.2%); this reduced to 32.7% following system consultation (McNemar test, p < 0.001). Subjects' mean 'unsafe' workups per case decreased from 0.49 to 0.32 (p < 0.001). System advice prompted the clinician to consider the 'correct' diagnosis (established at discharge) during initial assessment in 3/104 patients. Median usage time was 1 min 38 sec (IQR 50 sec-3 min 21 sec). Despite a modest increase in the number of diagnostic possibilities entertained by the clinician, no adverse effects were demonstrable on patient management following system use. Numerous technical barriers prevented subjects from accessing the diagnostic aid in the majority of eligible patients in whom they sought diagnostic assistance.

Conclusion: We have shown that junior doctors used a Web-based diagnostic reminder system during acute paediatric assessments to significantly improve the quality of their diagnostic workup and reduce diagnostic omission errors. These benefits were achieved without any adverse effects on patient management following a quick consultation.
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http://dx.doi.org/10.1186/1472-6947-6-37DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1654143PMC
November 2006

Long term morbidity and health related quality of life after multi-system Langerhans cell histiocytosis.

Eur J Cancer 2006 Oct 7;42(15):2563-9. Epub 2006 Sep 7.

Department of Haematology/Oncology, Great Ormond Street Hospital for Children, London, and Yorkshire Regional Centre for Paediatric Oncology and Haematology, St James University Hospital, Leeds, UK.

Background: Langerhans' cell histiocytosis, a clonal multisystem disorder, can affect children or adults resulting in long term sequelae. However, the overall morbidity for survivors has not been formally determined.

Patients And Methods: We performed a cross-sectional study of 40 unselected long term survivors of childhood multisystem Langerhans cell histiocytosis, involving clinical examination, health-related quality of life assessment, brain imaging, neuropsychometry, endocrine assessment, respiratory function tests and audiometry. A specific 'morbidity score' was devised to measure outcome.

Results: Seventy-five percent of patients had detectable long term sequelae, hypothalamic-pituitary dysfunction (50%), cognitive dysfunction (20%) and cerebellar involvement (17.5%) being the most common. Half had moderate to severe morbidity, and the worst-affected patients were unable to lead an independent adult life. Health-related quality of life, which correlated well with the morbidity score (p<0.001), was adversely affected in >50% of patients.

Conclusion: Organ damage from multisystem Langerhans cell histiocytosis causes long term morbidity extending into adult life. Carefully planned, multidisciplinary follow up is essential to ensure early recognition of problems with appropriate interventions to reduce the impact on patients' 'quality of life'.
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http://dx.doi.org/10.1016/j.ejca.2006.05.031DOI Listing
October 2006

Intraocular Langerhans cell histiocytosis in a neonate resulting in bilateral loss of vision.

Pediatr Blood Cancer 2006 Oct;47(5):633-5

Department of Oncology, Great Ormond Street Hospital for Children NHS Trust, and Department of Ophthalmology, University College Hospital NHS Trust, London, UK.

Intraocular involvement in Langerhans cell histiocytosis (LCH) is rare. We describe the case of a neonate with congenital disseminated LCH involving skin, liver, spleen, and intraocular structures including uvea and retina. Early and aggressive treatment according to the LCH-II treatment protocol was administered and resulted in remission of the disease. However, despite close follow-up and additional local treatment, involvement of intraocular structures resulted in severe long-term ophthalmological sequelae including complete bilateral loss of vision.
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http://dx.doi.org/10.1002/pbc.20714DOI Listing
October 2006

Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group.

Pediatr Blood Cancer 2004 May;42(5):438-44

Scientific Directorate and Department of Hematology/Oncology, Gaslini Children's Hospital, Genoa, Italy.

Background: Permanent consequences (PC) are often described among subjects with Langerhans cell histiocytosis (LCH) but data on the real incidence are scarce. Within the Histiocyte Society (HS), and in order to design a definitive late effects study, a retrospective survey was organized to describe the prevalence of PC among long-term survivors of LCH.

Methods: Nine institutions contributed with their LCH patients having a minimum follow-up of 3 years. Information was collected on their disease-history, and on type and date of onset of any PC. Because of the retrospective type of this study, it was accepted that each institution might have used different criteria to assess PC.

Results: One hundred eighty-two subjects were registered and in 95 (52%) at least 1 PC was reported. For some specific PC (e.g., anterior pituitary dysfunction) information was too scarce to provide reliable data. PC were more frequent among subjects with multisystem (MS) disease (71%), compared to those with single system (SS) disease (24%); P < 0.0001. The most frequently reported PC were diabetes insipidus (DI) (24%) orthopedic abnormalities (20%), hearing loss (13%), and neurological consequences (11.0%). Analysis of cumulative risk showed that some types of PC may become manifest more than 10 years from diagnosis.

Conclusions: This survey on selected cases of LCH survivors has confirmed that late sequels are frequent, and that they are even more common among those with MS LCH. Our findings highlight the need for long-term and patient-oriented follow-up in children with LCH.
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http://dx.doi.org/10.1002/pbc.20021DOI Listing
May 2004

Cognitive outcome of long-term survivors of multisystem langerhans cell histiocytosis: a single-institution, cross-sectional study.

J Clin Oncol 2003 Aug;21(15):2961-7

Department of Hematology/Oncology, The Institute of Child Health and Great Ormond Street Hospital, London, United

Purpose: Damage to the CNS, including the cerebellum, and to the hypothalamopituitary axis, is documented in Langerhans cell histiocytosis (LCH). Neuropsychologic deficits have been recognized, but this is the first study in which cognitive function has been systematically assessed in a cohort of patients.

Patients And Methods: Twenty-eight long-term survivors of multisystem LCH (mean age, 15.1 years) were investigated for intelligence, memory and learning, language, and academic attainments.

Results: The mean intelligence quotient (IQ) of the entire group was not significantly different from the mean of the population (ie, mean +/- SD, 100 +/- 1), but there were wide ranges (Full-Scale IQ [FSIQ]: mean, 93.6; range, 61.7 to 134; Performance IQ [PIQ]: mean, 92.2; range, 46 to 136; and Verbal IQ [VIQ]: mean, 93.7; range, 64.2 to 126). CNS involvement was a significant risk factor for lower scores, but sex, diabetes insipidus, and cranial radiotherapy were not. The CNS group had lower VIQ, PIQ, and FSIQ than patients with no CNS involvement (no CNS group: mean +/- SD FSIQ, 102.3 +/- 15.6; CNS group: mean +/- SD FSIQ, 73.6 +/- 7.7; P <.001). A similar pattern of results was obtained for all other cognitive measures. Even when effects of reduction in FSIQ were taken into account, specific deficits were found in patients in the CNS group.

Conclusion: Long-term survivors of multisystem LCH, particularly patients with CNS involvement, may develop significant cognitive deficits. All patients should have formal, repeated neuropsychologic assessment as part of long-term follow-up, which will enable abnormalities to be detected early so that appropriate supportive measures can be offered.
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http://dx.doi.org/10.1200/JCO.2003.05.048DOI Listing
August 2003