RESUMEN
Rubber band constriction syndrome has been described in the literature, although there are very few case reports. Non-healing recurrent tenosynovitis and synovitis of the wrist joint demonstrating a circular rubber band on imaging has not been described before. Imaging studies showed retained circular band deep to the extensor tendons, embedded within the joint capsule. Surgical removal of the band by open incision led to a dramatic improvement in the outcome of the patient. Level of evidence V.
RESUMEN
Aggressive systemic mastocytosis (ASM) is characterized by mast cell accumulation in systemic organs. Though ASM may be associated with other hematological disorders, the association with pure red cell aplasia (PRCA) is rare and has not been reported. Pure red cell aplasia (PRCA) is a syndrome, characterized by normochromic normocytic anemia, reticulocytopenia, and severe erythroid hypoplasia. The myeloid and megakaryocytic cell lines usually remain normal. Here, we report an unusual case of ASM, presenting in association with PRCA and the management challenges.
RESUMEN
Spur cell haemolytic anaemia (SCA) is a form of anaemia that can be seen in patients with severely impaired liver function or advanced cirrhosis. It is associated with high mortality. The treatment options for SCA secondary to cirrhosis are limited. Our patient is a middle-aged man who developed SCA and was not a candidate for liver transplantation or splenectomy. High-dose steroids helped ameliorate haemolysis and improve anaemia and general condition of our patient.
Asunto(s)
Anemia Hemolítica Autoinmune/tratamiento farmacológico , Glucocorticoides/administración & dosificación , Glucocorticoides/uso terapéutico , Metilprednisolona/uso terapéutico , Esteroides/administración & dosificación , Esteroides/uso terapéutico , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/fisiopatología , Lesión Renal Aguda/terapia , Anciano , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/fisiopatología , Mareo , Humanos , Hepatopatías/diagnóstico , Hepatopatías/fisiopatología , Hepatopatías/terapia , Masculino , Metilprednisolona/administración & dosificación , Enfermedad del Hígado Graso no Alcohólico/fisiopatología , Resultado del TratamientoRESUMEN
Cholangiocarcinomas (CCA) are uncommon malignancies that present late and have a poor prognosis. The patients may remain undiagnosed for many years because of non-specific presentation. CCA metastasises commonly to liver and adjacent lymph nodes. It can rarely metastasise to bone, lung, colon, pancreas, adrenal glands and spleen. Multiple treatment options are available including surgery, chemotherapy, radiotherapy and photodynamic therapy. The tumours have high rate of recurrence and most patients require palliative care. Our patient is a middle-aged man who presented with pain in right ring finger, workup of which revealed digital metastasis from underlying cholangiocarcinoma.
Asunto(s)
Neoplasias de los Conductos Biliares/diagnóstico , Neoplasias Óseas/diagnóstico , Colangiocarcinoma/diagnóstico , Neoplasias de los Conductos Biliares/diagnóstico por imagen , Neoplasias de los Conductos Biliares/patología , Neoplasias Óseas/complicaciones , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/secundario , Colangiocarcinoma/diagnóstico por imagen , Colangiocarcinoma/secundario , Diagnóstico Diferencial , Resultado Fatal , Dedos , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Dolor/etiología , Costillas , Tomografía Computarizada por Rayos XRESUMEN
Non-cirrhotic, non-malignant portal vein thrombosis (PVT) is commonly secondary to inherited or acquired prothrombotic states. However, even after extensive workup, 25% of patients with PVT have no apparent prothrombotic aetiology identified (idiopathic PVT). Inherited conditions include factor V Leiden, PT mutation and protein C/S/AT deficiency. Acquired conditions include APS, PNH and BCR-ABL 1-negative myeloproliferative neoplasms (MPN). BCR-ABL-1 negative MPNs are the most frequent underlying prothrombotic risk factor for PVT (15%-30%). However, peripheral blood counts often remain within normal ranges in these patients with MPN because of portal hypertension sequel. Despite suggestive features of MPN in bone marrow, these patients lack adequate diagnostic criteria and are classified as occult MPN. The discovery of recurrent molecular abnormalities such as CALR gene exon 9 mutation presented a crucial advance in the diagnosis of occult MPNs. In our patient, the diagnosis of MPN was made on this basis, despite lack of peripheral evidence of MPN.