Chorea disclosing deterioration of polycythaemia vera

Abstract

Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described. (Postgrad Med J 2000;76:655-656) Keywords: pyrexia of unknown origin; cervical lymphadenopathy Case reportA 19 year old south Asian women was admitted with a six week history of fever, malaise, anorexia, and cervical lymphadenopathy. She had been well until onset of her current illness. She was on no medication and had no contacts with tuberculosis.She was born in India but had moved to the UK at the age of 9 years. Foreign travel included a brief return to her native country 10 years previously and a trip to the United States two years before presentation.Clinical examination revealed a young women who appeared well; her temperature was 39ºC. The main findings were several tender mobile posterior cervical lymph nodes of which one was large. There were no lymph nodes elsewhere and she had no splenomegaly. The rest of the clinical examination was unremarkable.Investigations revealed a low white cell count at 2.7 × 10 9 /l with a neutrophil count of 1.3 × 10 9 /l, a raised plasma viscosity of 1.99 cp (normal range 1.5-1.72). A biochemical profile, chest radiography, and abdominal ultrasound gave normal results.The clinical impression was that this was likely to be tuberculous as she was Asian, or it could possibly be a lymphoma. Her clinical course was of a swinging intermittent pyrexia of up to 40.5ºC. Blood and urine cultures, thin and thick films for malaria parasites, cytomegalovirus, Epstein-Barr virus, and HIV titres were all negative.Tests for antinuclear factor, double stranded DNA, and antineutrophil cytoplasmic antibodies were negative. Thyroid microsomal antibody titres were positive at 1/400 and T cell analysis showed severe T lymphopenia of CD4, CD8, and NK populations with T cell activation.Later in her illness she had computed tomography of her chest, abdomen, and pelvis which was unremarkable, although minimal splenic enlargement was noted. Bone marrow aspiration and a trephine biopsy did not show any evidence of lymphoma or non-caseating granulomas.The large posterior cervical lymph node was excised. Microscopic examination using an antibody against CD 68 showed numerous darkly staining histiocytes, many of them containing cell fragments. There was necrosis and apoptosis and immature lymphoid cells in keeping with a diagnosis of Kikuchi's disease. Kikuchi's disease was originally described in the Far East in 1972 and over the last decade has become increasingly recognised in the Western world.Antibiotic treatment was withdrawn. Her temperature settled after three weeks, but she continued to remain neutropenic for six weeks from the beginning of the illness. When reviewed in outpatients four weeks after discharge from hospital she was well and all her lymph nodes had disappeared. ...

Hemichorea in a patient with JAK2V617F blood cells

“…[4][5][6] The exact mechanism underlying PVC is not known and there are no specific neuroimaging abnormalities or pathologic findings. 7,8 Chorea in this patient represents a possible forme fruste of an MPN in that the patient's clinical features did not allow for a definitive diagnosis of PV even though she was JAK2V617F ϩ . A similar situation has been documented in patients who present with splanchnic vein thrombosis and are found to be JAK2V617F ϩ .…”

Corea y eritrocitosis de altura: reporte de caso

“…Posteriormente, han aparecido más publicaciones relacionando estas enfermedades, en las cuales la mayoría de los pacientes descritos fueron mujeres y la causa de eritrocitosis más frecuente fue la PV. Es conocido que los pacientes con PV cursan con manifestaciones neurológicas en un alto porcentaje (50-70%), las más frecuentes son cefalea, vértigo, parestesias, alteraciones visuales e infarto cerebral (8) . Se ha descrito, como complicación infrecuente, la presencia de un síndrome extrapiramidal en el 0,5-2,5% de pacientes con PV y es el síndrome coreico el más frecuente.…”

Chorea in the Older Adult: A Full Blooded Answer

“…due to chronic lung disease). 10 Interestingly, while PV has a slight predominance in males (2:1), 9 polycythaemia verarelated chorea occurs predominantly in females (4:1).…”

“…16 In patients with polycythaemia vera-related chorea, haloperidol and tetrabenazine (a presynaptic dopamine depleter) are typically given first line for symptomatic relief and can be withdrawn as symptoms improve. 8,10,17 Regular venesections are usually effective in controlling the symptoms of chorea, with the majority of cases resolving. Aggressive therapy is essential as the severity of chorea appears to be inversely related to the haemoglobin and haematocrit.…”