Pediatric Hematology Oncology Journal最新文献

Acute COVID-19 infection typically results in mild disease in children, but can lead to several immune manifestations. One such severe manifestation is immune-mediated thrombotic thrombocytopenia (iTTP). It is exceedingly rare in children, and only a few cases have been reported worldwide. We report a case of severe iTTP in a 17-year-old girl. She was positive for COVID-19 infection by reverse-transcription polymerase chain reaction at presentation and had received a Covaxin injection two weeks earlier. She responded to therapeutic plasma exchange, corticosteroids, and rituximab. Early recognition of this complication and prompt initiation of therapeutic plasma exchange and corticosteroids is paramount to achieving a good outcome in this rare, life-threatening complication.

Background

Afibrinogenemia is a rare disorder, with autosomal recessive inheritance, most often associated with consanguinity. To date, very few cases have been reported from India. The aim was to study the clinical heterogenicity of patients with afibrinogenemia.

Case series

We present a retrospective study of five patients with afibrinogenemia diagnosed at our institute. All five patients had bleeding as a primary symptom. The median age for the first episode of bleeding was 150 days (range: 2 days - 18 months). Four were born of third degree consanguineous couple. The male to female ratio was 0.66. One patient had thrombosis along with bleeding. All patients had normal platelet count with deranged prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time, and low to nil fibrinogen levels. All had at least one episode of deep bleeding. The sites of bleeding included: a) intracranial (n = 3), b) intramuscular (n = 1), and, c) hemarthrosis (n = 1).

Conclusion

Afibrinogenemia should be considered as a differential diagnosis in patients with bleeding from any site with prolongation of PT and APTT. Paradoxical arterial and venous thrombosis may be a manifestation, but is rare. Thrombotic phenotype requires management with fibrinogen infusion along with anti-coagulants.

In the last four decades, survival in childhood cancer in India has improved significantly, however we increasingly face the question that has challenged providers in high-income countries: when care becomes futile and merely prolongs suffering, how should that be addressed? This position statement was prepared by a panel in the Pediatric Hematology-Oncology chapter of the Indian Academy of Pediatrics (IAP-PHO), with external experts, using a process endorsed by the Executive Committee of IAP-PHO. The panel consisted of providers and stakeholders who were committed to improving end-of-life care (EOLC) in children with cancer. Three case scenarios of terminally ill children with cancer were presented, and the various challenges of EOLC in each situation were brought up, including families who wanted everything possible done for their child and the fear of medicolegal issues by physicians. We emphasize the importance of involving palliative care services early in the process to ensure that families receive a consistent message about their child's outcome. With the recent Supreme Court of India decision, there is now a viable EOLC pathway to withdraw life-sustaining therapy (LST) from children who are terminally ill. We outline it and discuss the various barriers to the withdrawal of LST. Increased access to palliative medicine services, which currently exist sporadically across India, is a core necessity. IAP-PHO hopes to actively work with organizations representing colleagues in palliative care and intensive care, to see how we can improve EOLC in childhood cancer.

Background

Secondary hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal condition commonly triggered by infections. HLH secondary to hepatitis E or hepatitis A and E coinfection has been scarcely reported. Diagnosing HLH in sickle cell anemia can be tricky.

Case report

We present a 10-year-old boy, a known case of sickle cell anemia, who presented with acute viral hepatitis (HAV and HEV IgM positive). He initially improved with supportive management. However, on the fifth day of illness, he developed fever, tachypnea, and pancytopenia. Inflammatory markers, including serum ferritin, triglycerides, and D-dimer were raised. Bone marrow examination revealed hemophagocytosis. A diagnosis of sickle cell disease with HAV and HEV coinfection with secondary HLH was made. The patient responded to steroids and was well on follow-up after 3 months.

Conclusion

HAV and HEV coinfection with a background of sickle cell anemia with secondary HLH is very rare. Diagnosis of HLH in sickle cell anemia is challenging. Timely diagnosis and management of secondary HLH are vital for a favorable outcome.