Hodgkin's lymphoma (HL) is a common type of lymphoma in children and adolescents, and it typically presents with lymphadenopathy, fever, and weight loss. Nephrotic syndrome is a rare but reported complication of HL, and it is associated with a poor prognosis. We report a case of a 12-year-old boy who presented with nephrotic syndrome as the initial manifestation of HL. The diagnosis was confirmed by a biopsy of the lymph node, which showed the characteristic Reed-Sternberg cells. The patient was treated with chemotherapy and he achieved complete remission of both HL and nephrotic syndrome. This report aims to raise awareness of the potential association between HL and nephrotic syndrome in pediatric patients, which can aid in early detection and prompt management.
Fibromatosis colli, a rare benign congenital mass of the sternocleidomastoid muscle, is mainly found in the neonatal age group, often at the age of 2 to 4 weeks of life. Also known as a pseudo tumour, its aetiology is still unknown and may occur due to injury of the sternocleidomastoid muscle in the last trimester of intrauterine life or during delivery. Frequently manifests as restriction of neck movement and enlarging neck mass at around 14-28 days after birth. Ultrasonography (USG) neck is the preferred diagnostic modality. Treatment is mainly conservative consisting of observation and stretching exercises. Surgical intervention is required in <10% of cases and consists of a tenotomy of the sternocleidomastoid muscle. We present a case report of fibromatosis colli in a 15-day-old female neonate with a history of birth trauma presented with a right neck mass and torticollis, diagnosed using high-frequency ultrasound. This condition regressed in the next few months while the patient was instituted only conservative treatment. Thus, signifying the importance of clinical diagnosis and differentiation from other causes of neck masses to prevent unnecessary investigation and overtreatment.
Castleman’s disease (CD), also known as Angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder first reported by dr. Benjamin Castleman in 1956. The estimated incidence rate is 5 to 25 per million person-years. Histologically, the CD can be classified as hyaline-vascular type, plasma cell type and mixed type. HHV-8-associated MCD (Multicentric CD) is most commonly diagnosed in HIV-infected or otherwise immunocompromised individuals. Co-occurrence of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes is known as POEMS syndrome. Treatment of iMCD (idiopathic CD) is challenging, and outcomes are poor because of no uniform treatment guidelines. The anti-interleukin-6 monoclonal antibody Siltuximab with or without corticosteroids is the preferred first-line therapy for iMCD.
Osteopetrosis, or marble bone disease, is a rare genetic metabolic bone disease initially described by Albers-Schönberg in 1904. Osteopetrosis includes a clinically heterogeneous group of conditions characterized by increased bone density due to a defect in bone resorption by osteoclasts. The osteoclasto-genesis as well as the osteoclastic activity may be distorted. Clinical symptoms of osteopetrosis vary greatly in their presentation and severity as the spectrum ranges from the neonatal onset with life-threatening complications to incidental findings of osteopetrosis on radiographs. Diagnosis is based on clinical and radiographic evaluation, confirmed by bone biopsy and genetic testing. Treatment depends on the symptoms and severity of the disease and requires a multidisciplinary team approach.
Background: Acute lymphoblastic leukemia (ALL) is a common pediatric malignancy, typically manifesting with symptoms of bone marrow and hematolymphoid organ infiltration. Although hepatic involvement in the form of hepatosplenomegaly is seen in nearly two-thirds of the patients, primary presentation as acute liver failure is rare. Clinical Description: A 12-year-old boy presented with complaints of abdominal distention with jaundice for the past 15–20 days, associated with transient fever of 6 days. Examination revealed pallor, icterus, and hepatosplenomegaly. A possibility of acute infectious or autoimmune hepatitis or hemolytic anemia was considered. Management: Liver function tests were deranged with hyperbilirubinemia with modest increase in transaminases and serum alkaline phosphatase along with deranged prothrombin time. The complete blood count showed pancytopenia with normal peripheral smear. Investigations for common infectious causes were noncontributory, as also those for autoimmune hepatitis. The direct Coombs test was also negative. In view of persistent pancytopenia with hyperbilirubinemia and hepatosplenomegaly, a bone marrow evaluation was done, which revealed B-cell ALL (B-ALL). Thus, the child was diagnosed with a case of ALL presenting with acute liver failure. The child was initially managed as a case of acute liver failure and on diagnostic confirmation, he was started on the B-ALL chemotherapy protocol as per his risk stratification with hepatic dose modification of chemotherapy. The child showed complete normalization of the liver functions by about 4 weeks of induction chemotherapy and achieved remission. Conclusions: A child presenting with acute liver failure may be having an underlying undiagnosed, clinically silent ALL. After ruling out common causes of acute liver failure, one must not forget the possibility of leukemic blast infiltration of the liver as a cause of liver failure. With early initiation of advanced chemotherapeutic induction regimens, there can be dramatic resolution of liver injury and remission of ALL.
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