Clinical features and outcome in paediatric newly diagnosed immune thrombocytopenic purpura in a tertiary care centre

Objective: The study aimed to demonstrate the pattern of clinical presentations and outcome of acute Immune Thrombocytopenia [ITP] in our Centre

Methods: A descriptive, observational study was conducted by collecting and analysing the data of 103 patients of acute ITP, ageing between 1-14 years, at The Children's Hospital, Lahore from January 2016 to December 2016. We collected the data regarding age, sex, clinical presentations, history of preceding viral infections, vaccination history, laboratory values, different treatment options used, and response to the treatment concerning complete response, partial response and poor responders. Statistical analysis performed by using IBM SPSS statistics version 20

Results: We retrospectively, reviewed total 103 patients cases. The median age, at the time of presentation, was 5 +/- 3.4 years while mean age was 4.5 +/- 2.9 years. The male to female ratio was 1.28:1. Mean platelet count on presentation was 7 x 109/L [range: 0-24]. Twenty three [22.3 percent], patients had the history of preceding illness. Bruises, petechiae, epistaxis and hematemesis remained the common presentations. Six [5.8 percent] patients showed spontaneous recovery while 97 [94 percent] patients received treatment for ITP. Overall, 71 [68.9 percent] showed a response after treatment. Sixty-two patients [59.22 percent] showed loss of response and received treatment again. Among these patients, thirty-four patients [33 percent] developed chronic disease

Conclusion: Majority of patients presenting to our tertiary care centre had severe acute ITP on presentation. After management and follow-up, almost 1/3 of the patients develop chronic disease hence the incidence of developing chronic disease remained high as compared to the other centers

Demographics and outcome in paediatric non-hodgkin lymphoma: single centre experience at the children hospital, Lahore, Pakistan

Objective: To describe the patient demographics and outcome analysis in paediatric non-Hodgkin lymphoma [NHL] patients. Study Design: An observational study. Place and Duration of Study: The Hematology/Oncology Unit of The Children's Hospital and Institute of Child Health, Lahore, from January 2012 till December 2014

Methodology: Demographics including age, gender, histopathology, stage and outcome data, in biopsy proven NHL patients were analyzed. Burkitts/B Cell and Diffuse Large B Cell lymphoma patients were treated with MCP 842 Protocol while T/B-cell lymphoblastic lymphoma [LL] patients were treated with EURO-LB 02 protocol

Results: Ninety-one patients were treated during the study period at CHL. Data was insufficient in 18 patients, so they were excluded from the study. Patients included were 73. Males were 53 [72.6%]. Thirty-seven [50.7%] were 5-10 years of age, and 22 [30.1%] 10-16 years old. Abdominal mass was the commonest presentation seen in 32 [43.8%], lymphadenopathy in 27 [37%], intussusception in 5 [6.8%], while intestinal obstruction, obstructive uropathy, nasopharyngeal mass, gastric mass, primary bone lymphoma, pericardial effusion, jaw swelling, cheek swelling and paraspinal mass present in one [1%] each. Histopathological subtypes consist of Burkitt's lymphoma [BL] in 32 [43.8%], B cell NHL in 10 [13.7%], lymphoblastic lymphoma [LL] in 26 [35.6%], diffuse large B cell lymphoma [DLBCL] in 2 [2.8%], and anaplastic large cell lymphoma [ALCL] in 1 [1.4%]. Sixty-seven [91%] presented in stage III, and six [8.4%] in stage IV. Forty-eight [65.8%] patients had completed treatment and are well to date, 16 [21.9%] died, 5 [6.8%] left against medical advice [LAMA], and 4 [5.5%] patients relapsed

Conclusion: Burkitt's lymphoma was the commonest type of NHL seen in this cohort that predominantly presented with an abdominal mass. Children usually presented in advanced stage with delayed diagnosis. Better supportive care can improve the prognosis significantly. Training of pediatricians is equally important along with increasing parental/family knowledge about the disease symptoms so that they can seek early medical care, and earlier diagnosis is possible

Paraneoplastic cushing syndrome due To Wilm's tumor

Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed

Familial hodgkin lymphoma

Hodgkin Lymphoma [HL] is a lymphoid tumour that represents about 1% of all neoplasms occurring worldwide. HL is the most treatable of childhood malignancies. The etiology of HL is unknown. However, increase risk has been reported in males, with autoimmune diseases, poor socioeconomic status, increased family size, Ebstein Barr Virus [EBV] exposure, congenital or acquired immunodeficiency and those with a family history of HL. Familial HL is rare. The risk of developing HL is increased six times in the siblings of the affected patients. Both genetic and environmental factors have been postulated in the pathogenesis. No case of familial HL has been reported in the literature from Pakistan. We report two families with familial HL occurring in siblings that have been successfully treated and are on our follow-up

Comparison of presentation and outcome in 100 pediatric Hodgkin lymphoma patients treated at children hospital, Lahore, Pakistan and royal Marsden hospital, UK

Objective: To compare differences in demographics and outcomes in childhood Hodgkin lymphoma [HL] presenting at the Children's Hospital Lahore [CHL], and Royal Marsden Hospital [RMH], UK

Study Design: An observational comparative study

Place and Duration of Study: From January 2011 to February 2012 at CH, Lahore and from October 2008 to February 2012 at RMH, UK

Methodology: Consecutive HL patients [50 from each hospital] were inducted. Data regarding age, gender, staging, histopathology and outcome were analysed. Clinical and pathological staging done according to Ann-Arbor and World Health Organization classification. Treatment duration was 6-8 months. They were followed for 6 months post-treatment. Frequencies of variables were noted and compared. Chi-square test was used for determining significance

Results: Patients from Children's Hospital, Lahore were younger [mean 7.9 years] with male predominance [n=42, 84%]. Histopathology showed Mixed Cellularity [MC] in 32 [64%], Nodular Sclerosis [NS] in 5 [10%], Lymphocyte Rich in 4 [8%] and lymphocyte depleted in 1 [2%], nodular lymphocyte predominant [NLP] in 1 [2%] each. Majority presented in stage IV [n=25,50%], or stage III [n=20,40%]. Constitutional B symptoms were present in 37 [74%]. Bone marrow involvement observed in 23 [46%]. Remission was achieved in 42 [84%] patients; 2 [4%] relapsed, 4 [8%] expired and 2 [4%] left against medical advice. In contrast, RMH patients were older [mean 11.8 years.] and 30 [60%] were males. NS [n=40,80%] and NLP [n=6,12%] types were predominant. Two [4%] patients were in stage I, 27 [54%] in stage II, 12 [24%] in stage III and 9 [18%] presented in stage IV. Fourteen [28%] had B-symptoms. None had bone marrow disease. Event free survival was 46 [92%]. Four [8%] patients relapsed. Three responded to second line therapy and one relapsed postautologous transplant

Conclusion: Significant differences were observed in age at presentation, stage, histopathology and extent of bone marrow involvement between the groups. Of interest is the bone marrow involvement in stage IV patients in Pakistan. Delayed diagnosis account for advanced stage but difference in pathological subtype needs further study

Ollier's disease with myelodysplastic syndrome

Ollier's disease also known as enchondromatosis is a rare skeletal disorder that is usually sporadic, non-hereditary, and characterized by abnormal bone development [skeletal dysplasia]. While this disorder may be present at birth [congenital]; it may not become apparent until early childhood with more obvious symptoms, such as deformities or improper limb growth. It carries high risk of skeletal, visceral and brain malignancy seen in approximately 25% of patients. Occurrence of Ollier's disease with myelodysplastic syndrome has never been reported in the literature. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow characterized by one or more cytopenias despite a relatively hypercellular bone marrow. We hereby report the case of a 14 years boy who presented with painless finger swelling and hepatosplenomegaly. Radiological and bone marrow findings confirmed the diagnosis of Ollier's disease with Refractory Anemia and Excess Blasts [RAEB-1]