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Medscape-Case Presentation Pediatric Non Hodgkin Lymphoma

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  Case Presentation pediatric Non Hodgkin Lymphoma History The presentation of patients with non-Hodgkin lymphoma is acute or subacute, in contrast to the indolent course that characterizes most lymphomas in adults. The duration of symptoms before diagnosis is generally 1 month or less, with specific complaints varying according to the predominant sites of involvement. Bone marrow involvement in non-Hodgkin lymphoma may cause generalized or migratory  bone pain, but clinically significant cytopenias are uncommon, with their presence suggesting a diagnosis of acute leukemia. Symptoms of localized disease Localized disease can manifest as lymphadenopathy (usually with firmness and no tenderness), tonsillar hypertrophy, or a mass in virtually any location. In children, however, non-Hodgkin lymphoma is primarily an extranodal disease. Patients with supradiaphragmatic disease (eg, lymphoblastic lymphoma) often report having a nonproductive cough, dyspnea, chest pain, and dysphagia. Abdominal tumors (usually small noncleaved cell lymphoma [SNCCL] or B-cell large cell lymphoma [LCL]) are associated with abdominal pain, constipation, masses, or ascites. An acute abdomen occasionally is observed and may be mistaken for appendicitis. Rare primary non-Hodgkin lymphoma of the pancreas presents with the clinical picture of pancreatitis.  [44]   Symptoms of large cell lymphomas Constitutional symptoms are uncommon in non-Hodgkin lymphoma, except in patients with anaplastic large cell lymphoma (LCL). Many of these patients have low-grade fever, malaise, anorexia, and/or weight loss. Because LCLs are biologically disparate, however, these lesions have a varied presentation that may include chest or abdominal complications. In rare cases, an LCL appears as an isolated bone lesion in association with pain, swelling, and a risk of  pathologic fracture. Patients with anaplastic LCLs sometimes present with painful skin lesions, bone lesions,  peripheral lymphadenopathy, and hepatosplenomegaly.  [45, 46]  The painful skin lesions may regress spontaneously. A finding less common than these is testicular, lung, or muscle involvement. Anaplastic LCLs may also result in an apparent cytokine storm, with fevers, vascular leakage,  and pancytopenia. Symptoms of CNS involvement Patients occasionally develop symptomatic CNS involvement before diagnosis. Headache, meningismus, cranial nerve palsies, and altered sensorium may be observed. Although CNS involvement is uncommon at the time of diagnosis, patients with non-Hodgkin lymphoma (particularly SNCCL) occasionally present with symptoms suggestive of meningoencephalitis. Other Among the less common lymphomas of childhood, primary cutaneous/subcutaneous involvement can be seen (eg, in cutaneous T-cell lymphoma or blastic plasmacytoid dendritic cell hematodermic neoplasm). Physical Examination Patients with non-Hodgkin lymphoma generally appear mildly to moderately ill. They occasionally have a low-grade fever. Patients may present with pallor, respiratory distress,  pain, and discomfort. A jaw or orbital mass is present in as many as 10% of patients in industrialized countries, but this finding is particularly common in African patients with endemic Burkitt lymphoma. Other clinical findings in non-Hodgkin lymphoma include the following:    Cervical or supraclavicular masses or adenopathy is/are firm, fixed, and nontender    Dyspnea or stridor may occur in patients with a mediastinal mass    In patients with superior vena cava syndrome, distended neck veins and plethora may be observed    Decreased breath sounds are secondary to bronchial obstruction or pleural effusion    Thoracic dullness to percussion may be present with pleural effusion.    Abdominal distention or a mass may be present with or without tenderness, rebound tenderness, and/or shifting dullness    Painful skin lesions suggest an anaplastic large cell lymphoma (LCL); the less common forms of cutaneous lymphoma (T-cell, blastic plasmacytoid dendritic) are typically nontender    Obtundation, agitation, and meningismus may be observed in individuals with CNS involvement.     Focal pain or swelling in the extremity may be present in patients with primary bone lymphoma. Relatively uncommon physical findings include the following:     Nasopharyngeal mass    Parotid enlargement     Nephromegaly    Testicular enlargement Diagnostic Considerations Conditions to consider in the differential diagnosis of non-Hodgkin lymphoma include the following:     Neuroblastoma    Rhabdomyosarcoma    Sarcoidosis    Toxoplasmosis    Tuberculosis    Wilms tumor Differential Diagnoses    Acute Complications of Sarcoidosis     Atypical Mycobacterial Infection     Cat Scratch Disease (Cat Scratch Fever)     Hodgkin Lymphoma     Intussusception     Lymphadenitis     Lymphoproliferative Disorders     Pediatric Acute Lymphoblastic Leukemia     Pediatric Acute Myelocytic Leukemia     Pediatric Appendicitis     Pediatric Mononucleosis and Epstein-Barr Virus Infection     Pediatric Neuroblastoma     Pediatric Rhabdomyosarcoma     Toxoplasmosis    Tuberculosis     Wilms Tumor    Approach Considerations A complete blood count (CBC) with differential and a platelet count in patients with non-Hodgkin lymphoma should be obtained to assess for possible involvement of the bone marrow and to determine the patient's transfusion requirements. Additional tests, listed in the next section, are run to assess the patient's renal and hepatic function and to monitor for possible tumor lysis syndrome. Lumbar puncture  Lumbar puncture with determination of the cerebrospinal fluid (CSF) cell count and differential is performed to assess CNS involvement, the presence of which alters therapy. Airway obstruction  Mediastinal or cervical lymphomas may cause airway compromise. The potential for respiratory arrest must be recognized, particularly if sedation or  general anesthesia is administered for a diagnostic procedure. Consider (1) administering local anesthesia alone for lymph node biopsy; (2) establishing a diagnosis with pleural fluid, peritoneal fluid, or bone marrow aspiration; and (3) performing local irradiation to stabilize the airway before obtaining a diagnostic specimen from a site outside the radiation field. Laboratory Studies As previously mentioned, a CBC with differential and a platelet count should be obtained in  patients with non-Hodgkin lymphoma to assess for possible involvement of the bone marrow and to determine the patient's transfusion requirements. Measure the prothrombin time, the activated partial thromboplastin time, fibrinogen, and the D-dimer level if the patient is febrile or if he or she has evidence of sepsis. The purpose is to assess for possible disseminated intravascular coagulation, which may require specific therapy. Obtain blood and urine cultures if the patient has a fever, especially if it is associated with neutropenia. If indicated, also obtain stool and throat cultures. Analyze the following to assess the patient's renal and hepatic function and to monitor for  possible tumor lysis syndrome:    Serum electrolytes    Blood urea nitrogen (BUN)    Creatinine    Uric acid
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