Classical Hodgkin's lymphoma with skeletal muscle involvement: a testing case

Skeletal muscle involvement in Hodgkin lymphoma is very rare. An 11-year male child presented with stage IV Hodgkin lymphoma and skeletal muscle involvement in right gluteal, piriformis and psoas muscles. He had resistant disease. He achieved remission with 3rd line chemotherapy, EPIC; and successfully underwent high dose chemotherapy with autologous stem cell rescue. The patient is well after 15 months of follow-up. Successful treatment signifies positron emission computed tomography [PET/CT] evaluation and multidisciplinary team discussions in diagnosis and management of a rare Hodgkin lymphoma presentation

Bone marrow involvement in metastatic pediatric ewing sarcoma

Objective: To evaluate the frequency of bone marrow involvement with metastatic lung and bone sites in newly-diagnosed pediatric patients with Ewing sarcoma [ES]

Study Design: An observational study

Place and Duration of Study: Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, from January 2010 to October 2015

Methodology: Newly-diagnosed pediatric-age patients with ES were inducted. Ten patients were excluded because bone marrow aspiration/biopsy [BMAB] was not done. Patients' medical records were reviewed for data collection of age, diagnosis, tumor volume, bone marrow diagnosis, metastatic work-up and outcomes

Results: A total of 139 patients with median age of 12 years were identified. The median volume of tumors was 529 ml. Eleven patients had bone marrow [BM] disease involvement. Five [45.5%] had bone metastatic disease and 1 [9%] had both pulmonary and bone metastases. Four patients [31.1%] with positive BM had primary limb disease

Conclusion: Ewing sarcoma patients with bone metastatic disease have a higher frequency of BM involvement. However, BM can be involved without metastatic disease. BMAB should still be considered at staging for newly diagnosed pediatric patients with localized ES

Acute lymphoblastic leukemia in a child with fanconi's anaemia

Fanconi anaemia [FA] is an autosomal recessive inherited disorder with progressive bone marrow failure, associated congenital malformation and solid and haematological malignancies. Acute myeloid leukemia is the commonest haematological malignancy followed by myelodysplastic syndrome in children with FA. FA transformed into acute lymphoblastic leukemia [ALL] is a rare phenomenon and one of the rarest haematological malignancies associated with this disorder. We are reporting a 13 years old girl with FA and positive chromosomal breakage. She required regular blood product transfusion. She was planned for haematopoietic stem cell transplantation [HSCT] but the sibling-matched donor was found to have chromosomal breaks as well. Later on, her peripheral smear showed blast cell. Bone marrow showed pre-B ALL. She was started on chemotherapy but died shortly due to complications of the treatment. For this rare condition conservative management is indeed essential, however, safe and appropriate chemotherapy regimen is needed

Aplastic anemia: clinicohaematological features, treatment and outcome analysis

To determine the clinicohaematological features, treatment and outcome of children diagnosed with aplastic anemia at a single institution. Observational study. The Aga Khan University Hospital, Karachi, from January 1999 till December 2008. Medical records of children aged less than 15 years of age diagnosed with aplastic anemia were reviewed. Clinicohaematological features, treatment and its response to therapy and outcome were recorded. Results were described in percentages. Ninety patients were diagnosed to have aplastic anemia [AA]; 65 were male during the study period. Age ranged from 1 to 15 years. Fever in 65 patients [72.2%], pallor in 53 [58.8%], skin bleeding in 49 [54.4%] and epistaxis in 31[34.4%] were the most common and frequent presenting features. Congenital [Fanconi's] anemia was found in 15 [16.6%] and acquired idiopathic in 75 [83.4%] of patients. Very severe aplastic anemia [VSAA] was seen in 29 [32.2%], 26 [28.9%] had severe AA and 17 [18.9%] had moderate AA. Eight patients [8.9%] underwent haematopoietic stem cell transplantation [HSCT], 12 [13.3%] received immunosuppressive therapy [IST] and 70 patients [77.7%] received other and supportive therapy. Five [62.5%] patients showed complete response to HSCT and 3 [37.5%] failed to engraft. IST showed complete response in 3 [25%], partial response in 5 [41.6%] and no response in 4 [33.3%]. Twenty two patients [24.4%] expired either due to infection in 16 [72.7%, fungal in 6, bacterial in 10] and intracranial haemorrhage in 6 [27.3%] cases. Majority of cases with AA were acquired and idiopathic in etiology. VSAA and SAA were frequent. Response to HSCT and IST was sub-optimal