Orthopedic disorders in children suffering from neuroblastoma with intraspinal extension: the experience of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology

Neuroblastoma (NB) is the most common extracranial tumor in children. In 5–15% of cases, the tumor extends into the spinal canal and can potentially cause neurological deficits and orthopedic problems that can develop both at the onset of the disease and at a later time. We analyzed data of 61 patients with NB and intraspinal extension who had been treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology over the period from Jan 2012 to Dec 2018. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. The treatment was delivered in accordance with the NB-2004 protocol. In all the children, magnetic resonance imaging and/or computed tomography of the CNS were performed to evaluate intraspinal extension of the tumor as well as the degree of spinal cord compression. The presence of scoliosis and its severity were determined at the baseline and at 2 years after the diagnosis using imaging data and Cobb angle measurement. Scoliosis was classified as mild if the Cobb angle was 10–25°, moderate if it was 25–40°, and severe if it exceeded 40°. In our study, 7/61 (12%) patients were diagnosed with scoliosis at the baseline assessment. The median age at diagnosis was 8.0 (2.3–11.8) months. The male to female ratio was 2.5:1. In 4/7 (57%) patients, the primary tumor was located in the retroperitoneum (outside the major organs), and in 3/7 (43%) patients – in the posterior mediastinum. In this group, 4/7 (57%) patients had INSS stage 2 or 3 tumors, 2/7 (29%) patients had stage 4 disease, and 1/7 (14%) had INSS stage 4S. The majority of patients (5/7 (71%)) were stratified into an observation group. In 6/7 (86%) patients, the tumor extended into the spinal canal involving the thoracic spine. In 6/7 (86%) cases, there was evidence of complete obstruction of the spinal canal. Neurosurgery was performed in 4/7 (57%) patients. All these patients were diagnosed with mild scoliosis at the baseline. At 2 years after the diagnosis, imaging data were available for 38/54 (70%) patients who had not had scoliosis at the baseline. This time, scoliosis was diagnosed in 9/38 (24%) cases. The median age at NB diagnosis was 8.2 (0.8–42.3) months, the male to female ratio was 2:1. In 7/9 (78%) patients, the primary tumor was located in the posterior mediastinum. The majority of patients were stratified into an observation group (7/9 (78%)). In 8/9 (89%) patients, the tumor extended into the spinal canal involving the thoracic vertebrae. In the majority of patients (4/9(44%)), the tumor filled 33 to 66% of the spinal canal. Neurosurgery was performed in 6/9 (67%) patients. In this group, 7/9 (78%) patients were diagnosed with mild scoliosis and 2/9 (22%) patients – with moderate scoliosis. NB with intraspinal extension can lead to various orthopedic problems including scoliosis that can be revealed both at the onset of the disease and at a later time, meaning that this condition requires a multidisciplinary approach involving orthopedic specialists.

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Fisher-Evans Syndrome in Children: an Analysis of Genetic Defects and Therapy Response

Pediatric Hematology/Oncology and Immunopathology ◽

10.24287/1726-1708-2019-18-3-41-45 ◽

2019 ◽

Vol 18 (3) ◽

pp. 41-45

Author(s):

Z. A. Kuzminova ◽

E. D. Pashanov ◽

A. V. Pavlova ◽

M. A. Kurnikova ◽

I. V. Mersiyanova ◽

...

Keyword(s):

Medical Research ◽

Single Case ◽

Disease Onset ◽

Research Center ◽

Evans Syndrome ◽

Genetic Investigation ◽

Female Ratio ◽

Pediatric Hematology ◽

National Medical ◽

Male To Female

The Fisher–Evans syndrome (FES), also known as Evans syndrome (ES) is an immune hemopathy characterized by bicytopenia or pancytopenia in the absence of other diseases. FES may be either primary/idiopathic, or secondary, occurring against the background of other diseases. In children, FES is frequently related to a primary immunodeficiency disorder (PID). This article presents a retrospective data analysis of a cohort of pediatric patients (n = 14) with FES, the aim of which is to demonstrate the necessity of conducting a molecular genetic investigation for PID diagnosis. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The patients from this cohort were presented at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology during the period 2012–2019. All of these patients underwent Next Generation Sequencing (NGS). The median age of disease onset was 5.5 years (from 5 months to 16 years). The male-to-female ratio was 1.8:1. Thanks to the results of the genetic investigation conducted on 14 patients with FES, it was possible to identify 10 children (71.4%) who had secondary FES. The median age of disease onset in patients with secondary FES was 4 years, the minimum age was 5 months, the maximum age was 12 years. The male-to-female ratio was 7:3. Most frequently, the disease onset was associated with ITP, immune thrombocytopenia (35.7% of patients) or simultaneously with ITP and AIHA, autoimmune hemolytic anemia (35.7% of patients); less frequently, the disease onset was associated with AIHA, with a subsequent occurrence of ITP (21.4% of patients). A pancytopenia was identified in 1 patient with FES associated with a PID. Among our patients with a disease onset associated with AIHA, there was not a single case with a PID. We noted an extremely low effectiveness of glucocorticoids and IVIgs for the treatment of FES: in practically 100% of cases, the patients in the analysed group required second and subsequent lines of therapy. Among other therapy types, effective for both primary and secondary FES, we should note rituximab (62% of patients stay in remission for up to 1 year) and MMF (80% of patients stay in remission for up to 1 year). On the basis of the conducted study we can conclude that male patients with FES under 18 years of age whose disease onset was associated with ITP need to be thoroughly tested for PID, including genetic testing, since patients in this group have the greatest PID incidence. Treating FES with glucocorticoids and IVIgs is nearly always not very effective and second and subsequent lines of therapy are required. The drugs of choice which are most effective for patients with FES are rituximab and MMF.

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Evans syndrome in children: the results of a retrospective study of 54 patients

Pediatric Hematology/Oncology and Immunopathology ◽

10.24287/1726-1708-2021-20-2-74-83 ◽

2021 ◽

Vol 20 (2) ◽

pp. 74-83

Author(s):

Z. A. Kuzminova ◽

A. V. Pshonkin ◽

E. V. Raikina ◽

A. V. Pavlova ◽

M. A. Kurnikova ◽

...

Keyword(s):

Genetic Testing ◽

Molecular Genetic ◽

Disease Onset ◽

Sustained Remission ◽

Evans Syndrome ◽

Female Ratio ◽

Pediatric Hematology ◽

National Medical ◽

First Line Therapy ◽

Male To Female

Evans syndrome (ES) is a rare disorder accompanied by autoimmune hemolytic anemia and immune thrombocytopenia. ES in children is often associated with the defect of immune system regulation. This article presents an analysis of the results of investigation of 54 children with ES admitted to the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology during the period 2012–2019. The male-to-female ratio was 1.07:1 and the median age of the disease onset was 4 years (0 months – 16 years). Based on the results of molecular genetic testing, the diagnosis was changed in three patients to congenital TTP. Thus, for further analysis 51 patients were available. Thirty-nine out of 51 children (76.5%) had secondary ES, the median age of the disease onset was 4 years (2 months – 12 years), the male-to-female ratio was 1.05:1. PID was genetically confimed in 25 out of 39 patients, in the remaining 14 cases the diagnosis of PID was based on clinical and laboratory fidings only. Twelve patients (22.2%) had idiopathic (primary) ES, the median age of the disease onset was 8 years (7 months – 16 years) and the male-to-female ratio was 1:1. The disease onset in ES was more often accompanied by isolated cytopenia: ITP – 22 (43.1%) patients, AIHA – 15 (29.4%) patients, immune neutropenia – 1 (2%) patient, simultaneously with AIHA and ITP – 8 (15.7%) patients, pancytopenia – 5 (9.8%) patients. The second cytopenia developed on average after 2 years (1 month – 9 years). Neutropenia was observed only in secondary ES. The mortality rate for ES was 9.8%. First-line therapy (IVIG, glucocorticoids) for ES showed low effctiveness and sustained remission was observed only in 5.9% of patients. Thirty-four (87%) patients with secondary ES and 9 (75%) patients with idiopathic ES required other lines of therapy. The next-line drugs that showed good effctiveness were rituximab (76.5%), MMF (94%), and sirolimus (83%). Combinations of rituximab and MMF, rituximab and sirolimus were also effctive and induced sustained remission without other therapy in 83.3% and 75% of patients respectively. Children with ES need thorough examination, including genetic testing, for the early diagnosis of PID and the exclusion of congenital TTP. The combination of rituximab and MMF proved to be the most effctive treatment for ES.

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Neuroblastoma with localization in the neck. Own experience Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Russian Journal of Pediatric Hematology and Oncology ◽

10.17650/2311-1267-2018-5-4-21-30 ◽

2019 ◽

Vol 5 (4) ◽

pp. 21-30

Author(s):

D. V. Shevtsov ◽

T. V. Shamanskaya ◽

D. Yu. Kachanov ◽

N. S. Grachev ◽

K. I. Kirgizov ◽

...

Keyword(s):

Medical Research ◽

Conflict Of Interest ◽

Risk Group ◽

Risk Groups ◽

High Risk Group ◽

Research Center ◽

Observation Group ◽

Pediatric Hematology ◽

Favorable Prognosis ◽

National Medical

Introduction.Neuroblastoma (NB) is the most common extracranial solid tumor in children. As a rule, NB is localized in the adrenal gland, retroperitoneal space and posterior mediastinum. The head and neck area belongs to the rare localization of NB, which accounts for 2.6 % of cases, and is most common in children aged 0–3 years. Localization of NB in the neck in most cases has a favorable prognosis.Materials and methods.For the period from September 2013 to September 2017 (48 months) in the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology received treatment for 8 patients with NB in the neck. Examination, assessment of the prevalence of the process and stratification into risk groups in all patients were carried out according to the recommendations of the protocol of the German group for the treatment of NB NB-2004. For the purpose of histological verification of the diagnosis and detection of unfavorable molecular genetic markers, patients underwent surgical intervention, performed risk-adapted therapy according to the NB-2004 protocol.Results.The median age of diagnosis was 8.7 (1.2–34.1) months. In our cohort of patients in 87.5 % of cases, the diagnosis was made in the first year of life. In most cases, there was not only the identification of tumor masses, but also other symptoms of the disease. In 3 (37.5 %) patients the 2nd stage was established, in 1 (12.5 %) patient – the 3rd stage, in 3 (37.5 %) patients – the 4th stage and in 1 (12.5 %) patient – 4S stage of the disease. When stratifying patients into risk groups, in the observation group and the high-risk group was stratified by 3 (37.5 %) children and 2 (25 %) patients were classified as high-risk group. 3 (37.5 %) patients showed unfavorable cytogenetic abnormalities. When evaluating the response to therapy in most patients, a complete and very good partial response was stated. Overall (OS) and event-free (EFS) survival rates were 75 ± 15 % and 50 ± 17 %, respectively. The median of observation is 43 (26–61) months.Discussion.NB with the localization of the primary tumor in the head and neck area is a favorable form in terms of the stage of the disease and the risk group, however, it should be noted that in our patient cohort half of the subjects showed the development of certain adverse events, which was also reflected in the OS and EFS. Moreover, this localization dictates its risks from the point of view of the surgical stage of treatment. The main danger is complications after surgical treatment associated with the anatomical proximity of the central arteriovenous trunks, cranial nerves, and their involvement in the tumor process. In the case of the development of life threatening conditions (LTC), it is possible to use low-intensity chemotherapy courses.Conclusion.Experience Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology shows the need for timely diagnosis and the start of treatment of NB with localization in the neck. The choice of management tactics in favor of carrying out only surgical treatment is possible in patients of the observation group without the development of LTC. Not always the localization of NB in the neck region correlates with a favorable prognosis.Conflict of interest. The authors declare no conflict of interest.Funding. The study was performed without external funding.

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Giant Benign Mediastinal Masses Extending into the Pleural Cavity

The Surgery Journal ◽

10.1055/s-0036-1584519 ◽

2016 ◽

Vol 02 (02) ◽

pp. e46-e50

Author(s):

Güven Sunam ◽

Murat Öncel ◽

Sami Ceran ◽

Kemal Ödev ◽

Hüseyin Yıldıran

Keyword(s):

Pleural Cavity ◽

Correct Diagnosis ◽

Posterior Mediastinum ◽

Exertional Dyspnea ◽

Female Ratio ◽

Neurogenic Tumors ◽

Mediastinal Masses ◽

The Mean ◽

Magnetic Resonance Imaging Mri ◽

Male To Female

Introduction The aim of the study was to evaluate the results of surgery to remove huge mediastinal masses and their pathology. Surgical resection was chosen for accurate diagnosis and treatment of the huge mediastinal masses extending into the pleural cavity. Methods Records were reviewed for eight patients who had the diagnosis of huge benign mediastinal masses and who underwent operation; details of the patients and operations were recorded. Results Mean age was 34.5 (range 22 to 44) years, and male-to-female ratio was 2:6. Computed tomography and magnetic resonance imaging (MRI) were used to evaluate the location and extent of the abnormality and to characterize the tissue components of the mass. Most of the tumors were located in the posterior mediastinum. The most frequent presenting symptom was exertional dyspnea. The majority of cases underwent posterolateral thoracotomy, and complete resection was possible in seven patients. Partial resection could only be performed in one. The mean diameter of the resected masses was 15 × 10 cm. Histopathologic examination revealed 3 neurogenic tumors, 2 teratomas, 1 thymolipoma, and 1 ectopic thyroid, and 1 hemangioma. Minor complication was seen in two cases. Conclusion The presurgical thoracic MRI provided correct diagnosis along with radiologic characterization and topography. Surgery must be the preferred treatment in huge benign mediastinal masses.

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Nodular lymphocyte-predominant Hodgkin Lymphoma in children. Retrospective clinical and morphological analysis of the patients. One Center experience

Pediatric Hematology/Oncology and Immunopathology ◽

10.24287/1726-1708-2021-20-2-111-120 ◽

2021 ◽

Vol 20 (2) ◽

pp. 111-120

Author(s):

M. A. Senchenko ◽

D. S. Abramov ◽

G. A. Nasirdinova ◽

E. V. Volchkov ◽

D. M. Konovalov ◽

...

Keyword(s):

Medical Research ◽

Hodgkin’S Lymphoma ◽

Statistical Significance ◽

B Cell Lymphoma ◽

Response To Therapy ◽

Hodgkin's Lymphoma ◽

Research Center ◽

Female Ratio ◽

Pediatric Hematology ◽

National Medical

Lymphocyte-predominant Hodgkin's lymphoma (NLPHL) is a unique variant of Hodgkin's lymphoma (LH) with a relatively good prognosis. The tumor differs markedly from classic LH and is one of the forms B cell lymphoma. Despite the indolent course, it has a tendency to multiple and often late relapses. Microscopically, the tumor has 6 distinguishable morphological patterns. Despite the prevalence in all age groups, most of the original studies were performed among adult patients, while there are only several publications among the children's population. The aim of this study – retrospective analysis pediatric group of the NLPHL, evaluate the prognostic implication of histopathologic variants. Сomparing our own data with another study groups. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Study was included the biopsies aged 3 to 18 years (median 10.5 years) of 28 patients with NLPHL from the archive by Department of Pathology Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology from 2014 to 2020. The tumor more commonly affects males (20 boys and 8 girls, male-female ratio, 2.5:1). Complete clinical information was available in 24 patients. The clonal molecular assays were performed in 2 cases of relapse/progression of the disease. The Fisher's exact test was used to compare and evaluate the statistical significance of the differences in groups of patterns. There were no significant differences between typical patterns and variants, probably due to the small number of the patients. Further research will create a predictive scale for stratification by the risk groups. In cases of poor response to therapy, there is a risk that the pattern will turn into a prognostically more unfavorable variant.

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Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

Scientific Reports ◽

10.1038/s41598-021-89844-7 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Qiaozhu Zeng ◽

Jingyuan Gao ◽

Xinyu Zhang ◽

Aichun Liu ◽

Zhenfan Wang ◽

...

Keyword(s):

Kidney Disease ◽

Clinical Laboratory ◽

Imaging Finding ◽

Age At Onset ◽

Female Ratio ◽

Related Disease ◽

Igg4 Related Disease ◽

Peking University ◽

Chinese Cohort ◽

Male To Female

AbstractWe aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD−) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People’s Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD− were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

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P129 The utility of routine operative swab culture in uncomplicated peri-anal abscesses

BJS Open ◽

10.1093/bjsopen/zrab032.128 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

Author(s):

Apoorva Khajuria ◽

Tuba Rahim ◽

Mariam Baig ◽

Kai Leong ◽

Apoorva Khajuria

Keyword(s):

Inflammatory Bowel Disease ◽

Perianal Abscess ◽

Female Ratio ◽

Incision And Drainage ◽

Common Presentation ◽

History Of ◽

Inflammatory Bowel ◽

Operative Findings ◽

The Cost ◽

Male To Female

Abstract Introduction Despite perianal abscess being a common presentation, certain aspects of its management remain controversial, especially the routine use of intra-operative swab cultures. Methods A retrospective review of patients that underwent incision and drainage procedures for a perianal abscess over a six-month period was undertaken. Results Over 6 months, 50 patients were identified. The male to female ratio was 3:1 and median ASA score was 1. Only 6/50 patients presented with recurrent abscess and 1 patient had history of inflammatory bowel disease. On the basis of operative findings, 39 patients (78%) had uncomplicated abscess (not associated with cellulitis, sinus or fistula); swab cultures were performed in 26 (67%) of these patients. All patients were discharged on the same day; microbiology reports did not impact the treatment and no patients were followed up in clinic post-operatively or presented with recurrence. The number of unnecessary microbiology swabs undertaken in this cohort equates to approximately 52 unnecessary swabs a year. The cost of one swab is £10.10p, which means £520 could potentially be saved annually. Conclusion Routine intra-operative swab cultures do not impact management decisions, add to unnecessary costs and therefore should not be undertaken in uncomplicated or first presentation of peri-anal abscesses.

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High rates of gastroesophageal cancers in patients with dyspepsia undergoing upper gastrointestinal endoscopy in Uganda

Endoscopy International Open ◽

10.1055/a-1480-7231 ◽

2021 ◽

Vol 09 (07) ◽

pp. E997-E1000

Author(s):

Ronald Mbiine ◽

Cephas Nakanwagi ◽

Olivia Kituuka

Keyword(s):

Positive Association ◽

Referral Hospital ◽

Outcome Variable ◽

Mulago Hospital ◽

Upper Gastrointestinal Endoscopy ◽

Female Ratio ◽

Mulago National Referral Hospital ◽

Male To Female ◽

Upper Gastrointestinal ◽

National Referral Hospital

Abstract Background and study aims Dyspepsia is the most common presenting symptom in the gastrointestinal clinic of Mulago National Referral hospital. The etiology is essentially not fully described in our patient population. This study was therefore conducted to establish the causes of dyspepsia based on endoscopic diagnosis among patients with dyspepsia seeking care at the National Referral hospital of Uganda. Patients and methods This retrospective study conducted in the endoscopy unit of Mulago hospital reviewed 356 patient endoscopy reports spanning January 2018 to July 2020 with a focus on those with a referral indication of dyspepsia. Age and sex were the independent variables of interest while the endoscopy findings as reported by the endoscopist were the outcome variable of interest. Results Of the 356 endoscopy reports reviewed, 159 met the inclusion criterion of dyspepsia as the indication. Participant mean age was 47.7 years (± 16.53) with the majority (25.79 %) in the fifth decade while the male to female ratio was 1. The majority of patients had organic dyspepsia (90.57 %) while the commonest finding was gastritis 69 (43.4 %). Gastroesophageal cancers represented (18) 11.32 % of all findings. There was a positive association between age > 50 years with gastroesophageal cancers (7.639) as well as age < 50 years and functional dyspepsia (2.794); however, all these were not statistically significant (P = 0.006 and (P = 0.095, respectively). Conclusions Organic/structural dyspepsia comprises over 90 % of investigated dyspepsia with 11 % comprising cancer among patients seeking endoscopy at the National Referral Hospital of Uganda.

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Astrocytomas of the spinal cord in children and adolescents

Journal of Neurosurgery ◽

10.3171/jns.1985.63.5.0669 ◽

1985 ◽

Vol 63 (5) ◽

pp. 669-675 ◽

Cited By ~ 135

Author(s):

Ronald Reimer ◽

Burton M. Onofrio

Keyword(s):

Spinal Cord ◽

Histological Grade ◽

Extended Period ◽

Spinal Deformities ◽

Duration Of Symptoms ◽

Female Ratio ◽

Content Type ◽

Orthopedic Procedure ◽

Male To Female ◽

Fine Print

✓ The authors review 32 cases of spinal cord astrocytoma in patients under 20 years of age who were treated at the Mayo Clinic between 1955 and 1980. There was a 1.3:1 male to female ratio. Twenty patients were between 6 and 15 years of age at the time of diagnosis. The duration of symptoms prior to definitive diagnosis varied from 5 days to 9 years, with an average of 24 months. The most common symptoms were pain (62.5%), gait disturbance (43.7%), numbness (18.8%), and sphincteric dysfunction (18.8%). The most common neurological findings were a Babinski response (50.0%), posterior column sensory dysfunction (40.6%), and paraparesis (37.5%). A median follow-up period of 8.6 years (range 0.8 to 25.5 years) revealed that the survival time diminished with increased histological grade of the astrocytoma (p < 0.001). The development of postlaminectomy spinal deformities represented a serious postoperative complication. This occurred in 13 patients and was first recognized between 8 and 90 months postoperatively. Six deformities occurred following cervical laminectomy, and eight patients required at least one orthopedic procedure. It is crucial to follow these patients for an extended period of time to watch for postoperative spinal deformities.

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Clinicohematological Study of Thrombocytosis in Children

ISRN Hematology ◽

10.1155/2014/389257 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 9

Author(s):

Nathiya Subramaniam ◽

Suneel Mundkur ◽

Pushpa Kini ◽

Nalini Bhaskaranand ◽

Shrikiran Aroor

Keyword(s):

Platelet Count ◽

Myeloid Leukemia ◽

Prospective Observational Study ◽

Myelogenous Leukemia ◽

Distribution Width ◽

Female Ratio ◽

Reactive Thrombocytosis ◽

Acute Myelogenous ◽

Male To Female ◽

Primary Thrombocytosis

Introduction. Primary thrombocytosis is very rare in children; reactive thrombocytosis is frequently observed in children with infections, anemia, and many other causes. Aims and Objectives. To identify the etiology of thrombocytosis in children and to analyze platelet indices (MPV, PDW, and PCT) in children with thrombocytosis. Study Design. A prospective observational study. Material and Methods. A total of 1000 patients with thrombocytosis (platelet > 400×109/L) were studied over a period of 2 years. Platelet distribution width (PDW), mean platelet volume (MPV), and plateletcrit (PCT) were noted. Results. Of 1000 patients, 99.8% had secondary thrombocytosis and only two children had primary thrombocytosis (chronic myeloid leukemia and acute myelogenous leukemia, M7). The majority of the children belonged to the age group of 1month to 2 years (39.7%) and male to female ratio was 1.6 : 1. Infection with anemia (48.3%) was the most common cause of secondary thrombocytosis followed by iron deficiency alone (17.2%) and infection alone (16.2%). Respiratory infection (28.3%) was the predominant infectious cause observed. Thrombocytosis was commonly associated with IDA among all causes of anemia and severity of thrombocytosis increased with severity of anemia (P=0.021). With increasing platelet count, there was a decrease in MPV (<0.001). Platelet count and mean PDW among children with infection and anemia were significantly higher than those among children with infection alone and anemia alone. None were observed to have thromboembolic manifestations. Conclusions. Primary thrombocytosis is extremely rare in children than secondary thrombocytosis. Infections in association with anemia are most commonly associated with reactive thrombocytosis and severity of thrombocytosis increases with severity of anemia.

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