Prognosis in aplastic anaemia: a review of 138 cases

The outcome of 138 patients with aplastic anaemia was studied to ascertain whether prognostic factors at the onset of the disease are predictive. Applying four classic prognostic criteria, two groups of patients were identified: (1) those with an absolute granulocyte count of less than 0.5 × 10⁹/l, platelet count less than 20 × 10⁹/l, reticulocyte count less than 15 × 10⁹/l, and non-myeloid marrow cellularity greater than 75% who had 100% mortality in the first three months from the onset of the disease, (ii) patients who fulfilled three or fewer of these criteria who had a 20% three month mortality. The survival of these groups differed significantly (P = less than 0.001). The variable that most closely correlated with prognosis was the number of non-haematopoietic cells in the bone marrow aspirate. When Lynch's prognostic formula for patients with aplastic anaemia was applied to the same group of patients, the correlation with prognosis was less close and the calculations were tedious. The identification of a group of patients with severe aplastic anaemia is important because these patients do not benefit from conventional forms of therapy and alternative approaches such as transplantation and immunosuppression are justified.     

Revisiting acquired aplastic anaemia: current concepts in diagnosis and management

Acquired aplastic anaemia is a rare, serious, immunologically mediated bone marrow failure syndrome, characterised by marrow hypoplasia of varying severity and significant pancytopenia. Careful attention and investigation, including molecular testing, is required to confirm the diagnosis and exclude other mimicking conditions, such as inherited bone marrow failure syndromes. In a proportion of patients, the disease evolves to myelodysplasia or acute myeloid leukaemia and in some there is an association with paroxysmal nocturnal haemoglobinuria. The disease has a major impact on patient quality of life. Haemopoietic stem/progenitor cell transplantation for eligible patients with an available donor is the only current curative therapy. Other patients may receive immunosuppression, most commonly with anti‐thymocyte globulin and cyclosporin. An initial response to immunosuppression is often encouraging, but relapse is common. Supportive care, including management of transfusion requirements and infections, is central to management. Promising new diagnostic tools and emerging therapies will likely transform approaches to this important, chronic and life‐threatening condition.     

Three cases of typical aplastic anaemia associated with a Philadelphia chromosome

We report three cases of typical aplastic anaemia (AA) associated with a Philadelphia chromosome. This translocation was detected at the time of diagnosis of AA (one patient) and when overt leukaemia was diagnosed (two patients: one chronic myeloid leukaemia and one acute lymphoblastic leukaemia) after AA therapy and recovery of blood counts. We discuss the literature arguments about considering some cases of AA as preleukaemic disorders and suggest that our cases illustrate the association of AA with a clonal malignant disorder. We conclude that cytogenetic analysis is necessary at diagnosis of AA or after recovery of blood counts.     

Extraparenchymal neurocysticercosis: report of five cases and review of management.

Neurocysticercosis due to parenchymal cysts carries a good prognosis regardless of therapy. Extraparenchymal neurocysticercosis (including ventricular, spinal, and subarachnoid types) carries a poorer prognosis. Most extraparenchymal cases present with hydrocephalus. Medical treatment alone in doses and schedules developed for parenchymal disease is frequently unsuccessful. For ventricular disease, most cases can be managed with shunting procedures either alone or together with the administration of antiparasitic agents (e.g., praziquantel or albendazole), without extirpation of the cysts. Subarachnoid disease was formerly associated with a case fatality rate of about 50%. However, with the combination of shunting procedures for hydrocephalus, antiparasitic agents, and, in some cases, surgical extirpation of the cysts, the prognosis is much improved. Spinal cysticercosis can be either leptomeningeal (which responds like subarachnoid disease) or intramedullary. For all forms of neurocysticercosis, the role of antiparasitic agents needs to be better defined.     

妊娠哺乳相关骨质疏松症5例并文献复习

目的通过5例妊娠哺乳相关性骨质疏松症(pregnancy and lactation-associated osteoporosis,PLO)病例报道并复习相关文献,提高临床医师对PLO临床特征及治疗方法的深入认识。方法收集2011-2017年就诊于中国人民解放军总医院的5例PLO患者的临床表现、实验室检查、治疗药物及随访结果,并汇总目前我国已报道的21例PLO患者的治疗转归资料。结果 5例PLO患者均为青年女性,平素月经规律,妊娠晚期及哺乳早期出现腰背痛及活动障碍,生化检查基本正常,影像学检查示椎体骨密度降低为主伴多发压缩性骨折。诊断后立即停止哺乳,给予钙剂、维生素D、降钙素及双膦酸盐治疗1～6个月后,腰背疼痛明显减轻。文献报道的21例PLO患者发病年龄22～40岁(平均30. 24岁),骨折距产后平均为2. 2个月,平均椎体骨折个数为4. 6个;治疗药物主要是钙剂、维生素D及双膦酸盐,双膦酸盐的治疗疗程为6～48个月(平均20. 8个月),进行3周到4年的随访,均结局较好。结论 PLO临床罕见,发病机制尚不明确,早期诊断并停止哺乳,给予钙剂、维生素D、降钙素、双膦酸盐等药物治疗,多数预后良好,但要长期随访。     

Pregnancy and severe aplastic anaemia: causal relation or coincidence?

The relationship between aplastic anaemia (AA) and pregnancy remains uncertain. To assess whether an association between pregnancy and severe aplastic anaemia (SAA) exists, we compared the frequency of pregnancy in 35 young women with newly diagnosed SAA with the expected frequency in the general population. The observed pregnancy rate in the SAA group was 3–6%. This percentage approximates the expected pregnancy rate of 4.4% in the general population and is not compatible with a strong association between pregnancy and SAA.     

Venous thrombosis associated with pernicious anaemia. A report of two cases and review

Since homocystinuria, an inborn metabolism error is complicated by venous thrombosis in about half of the patients, hyperhomocysteinemia has been suspected of favouring thrombosis. Several case-control studies and even meta-analysis have confirmed a link between venous thrombosis and mild hyperhomocysteinemia. Nevertheless, the proper role of homocysteine remains debated. Hyperhomocysteinemia is induced by vitamin B9 or B12 deficiencies in a vast majority of cases. The authors report on two patients with venous thrombosis revealing pernicious anemia and review nine similar cases found in the world literature. The literature concerning the relationships between homocysteine and venous thrombosis is briefly reviewed.     

Sarcocystis in man: a review and report of five cases.

Sarcocystis was identified in biopsy specimens of skeletal muscle from two adults in Singapore and one in Bombay, and in muscle obtained at autopsy from an adult in Uganda, and in the heart of a child in Costa Rica. Review of case reports revealed that in seven reported cases, including three reported by Lindemann, non-parasite objects were misinterpreted as sarcocysts; in four instances organisms of undetermined classification were described, and in 35 cases true sarcocysts were observed. Among the sarcocysts seen in the 40 cases (35 old, 5 new), seven morphological types were recognized, each representing one to several different species, all of which are zoonotic and none of which can be designated Sarcocystis lindemanni. Among the four types of sarcocysts found in skeletal muscle, three closely resembled a corresponding species found commonly in monkeys: one from a man in Uganda corresponding to a species in Ceropithicus talapoin, forms from India resembling one or two species in Macaca mulatta, and forms from Southeast Asia resembling a species in Macaca fascicularis. Among three types of sarcocysts found in the human heart, one resembled a species commonly seen in the heart of cattle. Of the 40 Sarcocystis infections in man, 13 probably were acquired in Southeast Asia, 8 in India, 5 in Central or South America, 4 each in Africa and Europe, 3 in USA, 1 IN China and 2 in unknown localities. Associated conditions include muscle soreness or weakness in 7 cases, subcutaneous swellings in 5, eosinophilia in 2, and periarteritis or polyarteritis nodosa in 2 cases. However, evidence of pathogenicity of the mature sarcocyst is inconclusive.     

Primary hepatic amyloidosis: a mini literature review and five cases report

Primary hepatic amyloidosis (PHA) is characterized by abnormal deposition of monoclonal immunoglobulin light chains (AL) in the liver. This rare condition is frequently undiagnosed or misdiagnosed and can be associated with poor prognosis. At present, the precise pathogenesis is not fully understood. Despite that hepatomegaly and elevated alkaline phosphatase (ALP) are present in most patients with PHA, no specific clinical markers have been identified. Staining of hepatic tissues with Congo Red is often regarded as the "gold standard". Pharmacological therapy should aim to rapidly reduce the supply of misfolded amyloidoge- nic AL. High-dose intravenous melphalan (HDM) and autologous stem cell transplantation (ASCT) appear to be the most appropriate therapy but controversies still exist.     

Primary thrombocythaemia associated with systemic mastocytosis: a report of five cases

Mastocytosis is an uncommon disease characterized by a proliferation of tissue mast cells involving various organs, particularly the bone marrow. Though rare, the association of mastocytosis and haematological malignancies is well established. However, the frequency of mastocytosis reported in patients with essential thrombocythaemia is relatively low. We report five cases of such an association. Our five patients were undergoing evaluation for thrombocyte when the bone marrow biopsy revealed the presence of mastocytosis. The pathogenetic significance of this association is poorly understood. The different hypotheses are discussed.     

Bone marrow transplantation for severe aplastic anaemia a review of the Westminster experience of 24 cases

Summary Twenty-four patients have received bone marrow transplantation for severe aplastic anaemia at the Westminster Hospitals since 1974. Twelve patients are long term survivors. Infectious complications in association with graft rejection, graft versus host disease or prolonged neutropenia were the major cause of death. In the last 18 months the introduction of more effective conditioning regimes and Cyclosporin A as graft versus host disease prophylaxis has improved the survival rate to 85%. One patient has required regrafting for late graft failure without evidence of graft rejection.     

头皮动静脉瘘五例并文献复习

目的 探讨头皮动静脉瘘的诊断方法及治疗特点.方法 回顾性分析新疆医科大学第一附属医院神经外科2015年4月至2021年8月连续收治的5例头皮动静脉瘘患者的病历资料,对其临床特点、治疗方式、预后等进行评价.采用临床症状结合影像学检查结果进行疗效评估.术后即刻行头颈部CT血管成像或DSA检查,并术后3个月经门诊或电话随访,...     

Hepatitis- associated aplastic anaemia:clinical characteristics and immunosuppressive therapy outcomes

<正>Objective To analyze the clinical characteristics and to evaluate immunosuppressive therapy(IST)response and survival in hepatitis-associated aplastic anemia(HAAA).Methods We retrospectively analyzed clinical characteristics,IST response,long-term survival and clonal evolution in 41 HAAA patients,and compared