Mei-Yoke Chan

Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma)

Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of cutaneous lymphoma in the pediatric population. It is characterized histologically by subcutaneous infiltration of pleomorphic cytotoxic T cells, mimicking a lobular panniculitis. Although usually described as having an indolent clinical course, the condition may be complicated by systemic involvement and hemophagocytic syndrome, resulting in a poorer prognosis. We present two pediatric patients with aggressive forms of subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome, and discuss the current literature.

Infantile lipofibromatosis of the upper limb

The imaging features of extensive lipofibromatosis presenting in a 1-day-old female infant are reported. This lesion involved her entire right upper limb, extending from the axilla to the palm of the hand. Radiographs showed marked deformity and thinning of all the right upper-limb bones due to pressure effect of soft-tissue enlargement, especially affecting the distal humerus and proximal forearm bones. Magnetic resonance imaging showed a huge soft-tissue mass infiltrating most of the muscles of the entire upper limb, with bony erosion. The mass was largely T1-isointense, moderately T2-hyperintense and showed marked enhancement. There were intra-lesional signal changes consistent with fatty elements. A lesion debulking procedure was performed and the histology was that of lipofibromatosis. The limb was found to be non-viable after the procedure and a subsequent above-elbow amputation was performed. Although the resection margins were not clear, she had no further recurrence over a subsequent 3-year follow-up period.

Novel deep intronic and missense UNC13D mutations in familial haemophagocytic lymphohistiocytosis type 3.

Odile Beyne-Rauzy Sabine Brechignac Sylvestre Marechaux Dominique Vassilieff Olivier Ernst C eline Berthon Emmanuel Gyan Marie-Pierre Gourin François Dreyfus Pierre Fenaux Christian Rose Service d’H ematologie et de cardiologie Hôpital Saint Vincent de Paul, Universit e Catholique de Lille, Lille, Service de m edecine interne, CHU Toulouse-Purpan, Toulouse, GFM, Groupe Francophone des My elodysplasies, Hopital Avicenne, Paris (AP-HP), Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris (AP-HP), Hôpital Cochin, AP-HP, Paris, Radiologie, Hôpital Huriez, CHU de Lille, H ematologie, CHU de Lille, Lille, , H ematologie, Hôpital Bretonneau, CHU de Tours, Tours, and CHU de Limoges, Limoge, France E-mail: rose.christian@ghicl.net

Bony presentations of childhood haematological malignancy to the emergency room.

Aim:  Bony complaints are well‐recognised symptoms of childhood haematological malignancy. However, this elusive symptom complex of haematological malignancy is not easily recognised in the emergency room (ER) where musculoskeletal problems are frequent. This study reviews bony complaints of childhood haematological malignancy in the ER.

Appendicitis in immunosuppressed children: Still a diagnostic and therapeutic dilemma?

Acute appendicitis in the immunosuppressed patients are often difficult to diagnose and are associated with increased morbidity and mortality. Recent debates on primary appendicitis and typhlitis‐associated appendicitis have contributed to the diagnostic and therapeutic dilemma. We retrospectively reviewed all our immunosuppressed patients with appendicitis. The most frequent presenting symptom was fever followed by non‐specific intermittent abdominal pain. The median delay in diagnosis was 4 days. CT scan was useful in making the diagnosis, but the presence of adjacent bowel thickening has contributed to the dilemma in diagnosis and treatment. Pediatr Blood Cancer 2008;50:1282–1283.

Langerhans cell histiocytosis - a mimicker of tuberculosis of the spine.

with paracetamol, while the treatment with carbamazepine improved her neurological impairment, which when associated to a dental conservative approach (use of a flat nipple to avoid the impact of the teeth against the tongue associated to the topical application of a chlorhexidine 0.5% and hyluronic acid-based gels), led to a clinical amelioration of the ulcer (Fig. 1d) with an improvement of feeding and a gradual gain of weight. RFD is a rare, oral, ulcerative condition described by Antonio Riga, an Italian physician in 1881. The oral ulcer is characterized by continuous traumatic injuries produced by natal or neonatal teeth, usually lower incisors, resulting in tongue ulceration. This form is called ‘precocious or early RFD’, and appears before 6 months of age, a period in which the child is edentulous. However, a ‘late RFD’ appearing in 6 months of age or in older children is also described. It is not due to a precocious teeth eruption, but, as in our case, to neurological or developmental disorders in a child with normal chronology of dental eruption. Hence, this latter disorder may be the initial presentation of some serious underlying medical problems, as Lesch–Nyhan syndrome, familial dysautonomia and/or other neurological disorders as parasomnia, hereditary chin trembling and facio-mandibular myoclonus that may cause nocturnal tongue biting. From a point of view of its clinical manifestation, the differential diagnosis of RFD includes granular cell tumour, myofibroma, eosinophilic granuloma and other traumatic ulcers. Malignancies (sarcoma and lymphoma) and infectious diseases (acquired/congenital syphilis, tuberculosis and ulcerative fungal infections) must also be ruled out. Because the precocious extractions may have a negative impact on the dentoalveolar growth of the children, the treatment of choice of RFD should be conservative (as in our case), reserving the surgical approach, with the extraction of the teeth and subsequent application of a space maintainer, only in case of non-healing in conservative treatment.

Paediatric Palliative Care in Singapore

Paediatric palliative care is the “Cinderella” of the palliative movement in the world. It is lagging behind adult palliative movement for a variety of reasons. In Singapore, the situation is no different: the adult palliative services are more developed and more accepted by the medical community as well as the public. When it comes to paediatric palliative services, there is a denial of its necessity and a lack of resources and support. However, the winds of change are here and there is currently a formal Paediatric Palliative Care Programme in the only children’s hospital in Singapore, the KK Women’s and Children’s Hospital. More funding is in the pipeline and the programme is set to expand. Paediatric palliative care has its own unique challenges and barriers which may make it difficult for adult services to serve paediatric palliative needs. There are many misconceptions about what palliative care is about and what role it should play in paediatric care, even amongst doctors. Hopefully with increasing awareness and better availability of services, and best of all, when the end-users (physicians as well as patient and family) experience the added value of palliative care, there would be better acceptance and utilisation of paediatric palliative services so that it becomes a necessary part of basic holistic health care for children with life-threatening or life-limiting illnesses.