Iron Deficiency Anemia Associated With Acid-Modifying Medications: Two Cases and Literature Review

Iron deficiency anemia is often listed among potential adverse effects of gastric acid-suppressive medications, given that gastric acidity promotes intestinal absorption of nonheme iron. Additionally, the antacid calcium carbonate can inhibit iron absorption. However, there is little direct clinical evidence that proton-pump inhibitors, histamine-2 receptor antagonists, or calcium carbonate cause iron deficiency anemia. Most case reports have had substantial limitations (e.g., minimal follow-up and presence of other causes of iron deficiency), and retrospective cohort studies have lacked sufficient patient-specific detail to make strong causal inferences. We present 2 cases—both with detailed, prospective 10-year follow-up—in which combinations of proton-pump inhibitors, histamine-2 receptor antagonists and calcium carbonate were clearly associated with development of iron deficiency anemia. Overt iron-deficiency anemia is probably uncommon in patients who use acid-modifying medications and who have no other conditions that predispose to iron deficiency. Nevertheless, clinicians should be aware of this potential complication, given widespread use of these agents.     

24例重型再生障碍性贫血患者抗胸腺细胞球蛋白联合环孢素治疗的疗效分析

目的：分析重型再生障碍性贫血（再障）患者采用ATG和环孢素联合免疫抑制治疗（IST）的疗效。方法：回顾性分析使用IST的24例重型再障患者临床资料,并随访8～79个月,分析IST的疗效及可能影响疗效的因素。结果：使用IST方案总体有效率达75.0%。对IST治疗有效的患者,白细胞恢复时间为15～42d,中位恢复时间为24d。获得治愈的6例患者,血小板恢复时间为2～6个月。合用雄激素的患者治疗反应率为82.4%,高于未合用雄激素患者的57.1%。＋30d时中性粒细胞绝对值（N）≥1.5×109/L的患者有效率为86.7%,N〈1.5×109/L患者的有效率为50.0%。有18例患者拟诊为真菌感染,1例合并浸润性肺结核。＋30d时N〈1.5×109/L的8例患者均出现典型的肺部真菌感染灶。结论：IST可有效减少输血依赖。合用雄激素可增加治疗反应率;治疗＋30d中性粒细胞绝对值≥1.5×109/L可能提示患者对IST反应好。中性粒细胞减少时间长的患者,感染发生率高,尤其是真菌感染。     

Pregnancy Associated Aplastic Anemia--A Series of 10 Cases with Review of Literature

Introduction: Pregnancy induced aplastic anemia is a rare entity and the association is not well explained. There are approximately 80 cases in the literature and we are presenting the largest series, so far, of 10 cases.

Results: Total of 10 cases had 11 pregnancies. Mean age at presentation was 25.45 years and mean gestation when symptoms first developed was 17.09 weeks. Pallor and bleeding manifestations were the most common presenting complaints. Mean Hb, TLC, ANC and platelets were 4.97 g/dl, 2.74×10⁹/l, 1.11×10⁹/l and 41×10⁹/l, respectively. Bone biopsy cellularity ranged from <5 to 25%. Nine out of 11 (81%) pregnancies were successful of which 7 was full term and 2 were premature. Two babies were small for dates. One spontaneous abortion and one intra uterine death (IUD) were observed. Two out of 11 mothers died due to disease after delivery. Two of the 8 surviving mothers, had spontaneous partial response (22%); 4 mothers were asymptomatic after therapy with immunosuppressives given for 6 months and 3 were lost to follow up without response. Specific therapy (cyclosporin) was tried in two mothers antenatally with partial response in one. One child whose mother was given cyclosporin antenatally had jejunal atresia at birth.

Conclusion: Pregnancy associated aplastic anemia is a rare association. Spontaneous remission can occur in 25-30% of patients. In the first trimester patients, pregnancy can be terminated while in advanced pregnancy patients can be followed up with stringent supportive care. Cyclosporin may be a safe drug antenatally in such patients. Patients with established aplastic anemia should avoid pregnancy.     

Pyridoxine-Responsive Anemia: Report of 2 Cases in Brothers and a Review of the Literature

The clinical and laboratory findings in two brothers with severe anemiaare presented. These findings were very similar in both cases. Evidence thatat least one of them suffered from a pyridoxine-responsive anemia is presented.It was assumed that the other had the same disorder since, in addition to thestriking similarity in the clinical and laboratory abnormalities, the latter’s anemia disappeared completely with the parenteral administration of vitamin Bcomplex, which provided him with 2 mg. of pyridoxine daily. These patientshave not relapsed 1.5 and 6 years after stopping the therapy.

A review of the reported cases with anemia responding to vitamin B₆ administration is presented. Common factors observed in some of these casesare: (1) severe anemia, microcytic and hypochromic in type, chiefly due to astriking maturation erythroblastic arrest at the basophilic stage with no defectin leukocytes or thrombocytes; (2) hyperferremia and hemosiderosis; (3) anabnormal tryptophan-loading test; and (4) complete or almost complete correction of all abnormalities with administration of vitamin B₆, even at smalldoses. These data were considered to suggest that the patients had a true deficiency in pyridoxine.

In other patients, no increased excretion of xanthurenic acid has been observed after a tryptophan-loading dose, and pyridoxine administration hasimproved the anemia only partially and has not reversed the serum iron parameters to normality. All of these other patients suffered from a familial hypochromic anemia not due to iron deficiency or from a "sidero-achrestic" or "refractory normoblastic" anemia.

The cause of the disorder in patients in the first group is unknown, but, byexclusion, increased requirement of vitamin B₆ seems to be the most likelypossibility.

Submitted on November 22, 1960 Accepted on February 14, 1961     

Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature

Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.     

Severe Hypophosphatemia Associated with Gallstone Pancreatitis: A Case Report and Review of the Literature

Severe hypophosphatemia (<1.0 mg/dl) is a rarely reported complication of acute pancreatitis; when it does occur, it is typically attributed to alcohol abuse rather than the pancreatitis itself (1–5). In the literature, pancreatitis is not cited as a cause of hypophosphatemia (5, 6–16). Both pancreatitis and hypophosphatemia have widespread ramifications on human physiology, affecting hematologic, neural, hepatic, endocrine, respiratory, and renal systems. Given the possible synergistic consequences of pancreatitis and low serum phosphate, we emphasize the importance of recognizing hypophosphatemia as a complication of pancreatic inflammation.Herein, we report a case of acute pancreatitis unrelated to alcohol abuse associated with severe hypophosphatemia and review the pathophysiology.     

组织胞浆菌病四例报告并文献复习

目的探讨组织胞浆菌病的临床特点、诊断、鉴别诊断及治疗。方法报道我院2008年12月—2009年7月由病理诊断确诊的4例组织胞浆菌病患者,并结合文献探讨该病的临床特征及诊治方法。结果对该病认识不足,通过组织病理活检诊断该病。结论组织胞浆菌病临床表现多样,骨髓象检查或组织病理学检查有确诊价值,使用两性霉素B等抗真菌药物治疗有效。     

恶性胸腺瘤并红细胞增多症1例及文献复习

目的:提高对恶性胸腺瘤合并红细胞增多症的认识。方法:报告1 例恶性胸腺瘤并红细胞增多症,并且复习有关文献。结果:该病十分罕见。其发病机制与恶性肿瘤分泌促红细胞生成素有关,也可能是胸腺瘤所致T细胞免疫功能紊乱所致。结论:真性红细胞增多症的诊断必须排除原发病因,特别是中老年患者,一定要常规筛查胸、腹等各部位的肿瘤,以免漏诊。     

克罗恩病合并严重骨质疏松症病例报道及文献复习

目的:探讨克罗恩病(CD)合并严重骨质疏松症的机制、临床表现及诊治方法,以提高临床医师对本病的认识及诊治水平.方法:报道1例21岁女性患者,因发热、腹痛、腹泻、便血诊断为CD,使用糖皮质激素(GC)治疗半年,剂量相当于泼尼松15～50 mg/d,患者逐渐出现双手关节、胸背及双侧肋骨疼痛,活动困难.行骨骼X光片检查及双能...     

Intensive Immunosuppression Therapy for Aplastic Anemia Associated with Dyskeratosis Congenita: Report of a Case

Dyskeratosis congenita (DC) is a very rare inherited disorder characterized by skin pigmentation, nail dystrophy, and mucosal leukoplakia. It is also associated with a variety of noncutaneous abnormalities, such as fatal pulmonary complications, malignancy, and bone marrow failure.We report the case of a 32-year-old man with DC associated with severe aplastic anemia (SAA). The traditional treatment of DC-associated SAA is allogeneic hematopoietic stem cell transplantation (HSCT). However, in this case, an HLA-matched donor was not available. Therefore our patient was given intensive immunosuppressive therapy with antilymphocyte globulin (ALG) and cyclosporine A (CsA). The hemogram findings improved after the treatment, but the patient died of pulmonary complications after being in stable condition for 6 months. The results support the possible use of intensive immunosuppression with ALG and CsA for DC-associated SAA as an alternative treatment for patients who are not eligible for HSCT.     

Irreversible Homonymous Hemianopia Associated with Severe Hyperglycemia and Cerebral Hyperperfusion: A Case Report and Literature Review

A 68-year-old man was admitted to our department because of left incomplete homonymous hemianopia accompanied by hyperglycemia. Both T2-weighted and diffusion-weighted imaging revealed a low signal intensity along the subcortex and high signal intensity along the cortex on the right parietal and occipital lobes. Furthermore, arterial spin labeling and single-photon emission computed tomography showed hyperperfusion at the right parieto-occipital lobe. However, the electroencephalography result was normal. Hyperperfusion improved after controlling the blood glucose levels; nevertheless, homonymous hemianopia remained. We suspect that the irreversible brain damage was attributable to hyperperfusion associated with long-term hyperglycemia.     

Persistent Questions about the Treatment of Severe Aplastic Anemia in Children as Illustrated by Five Cases

We present our recent experience treating five children with severe aplastic anemia (SAA). One patient was transplanted and the remaining 4 patients were treated with immunosuppression of various types and duration. These five cases illustrate the many outstanding points which remain to be clarified regarding the care of children with SAA. We have outlined the treatment options available to each of these patients and the path that we followed along with their outcomes. Additional research and consideration is necessary to arrive at definitive answers to the questions posed by these five cases.     

Castleman病:文献复习及6例报告

目的 :提高对Castleman病的认识。方法 :报告 6例Castleman病并复习有关文献。结果 :该病临床表现取决于受累的部位。例 1和例 2以表浅淋巴结肿大为其临床表现 ;例 3和例 4以肾区不适为其主诉 ,早期诊断困难 ;例 5和例 6为中心性Castleman病 ,临床表现复杂 ,早期均误诊为结缔组织病 ,分别在起病 2年和 3年后才确诊。结论 :重视淋巴结活检是早期诊断的关键     

Hepatic Schistosomiasis: Report of Two Cases and Literature Review

Two Filipino patients were referred for evaluation of abnormal liver enzymes. Both patients were known to come from regions endemic for Schistosoma japonicum. The clinical laboratory and radiological features of these patients are presented. We highlight the findings of radiologic evaluation, including magnetic resonance imaging (MRI). The epidemiology, clinical manifestations, diagnostic strategies including a comparison of the utility of ultrasound, computed tomography, and MRI are reviewed. The pathophysiology responsible for hepatic manifestations of schistosomiasis is reviewed. An increased influx to the United States of persons from regions endemic for schistosomiasis, such as South America, Southeast Asia, and the Philippines, will result in an increase in the prevalence of the disease in this country. The diagnosis of hepatic schistosomiasis will need to be considered in populations at risk for contracting this common worldwide problem.     

Anal Tuberculosis: Report of Two Cases and Literature Review

Tuberculous involvement of the anus is an extreme rarity. We report two cases of anal tuberculosis and discuss the clinical features, the difficulty in differentiating from Crohn's disease, and the diagnostic aspects in the context of the pertinent medical literature.     

Case Report of Unilateral Eosinophilic Fasciitis in a Vietnamese Woman

Symmetric skin thickening of the limbs with deep fascial inflammation is the hallmark of eosinophilic fasciitis. We describe a woman who presented with unilateral progressive skin thickening. Examination of a full-thickness skin biopsy revealed an inflammatory process and fascial changes consistent with eosinophilic fasciitis. In contrast to other scleroderma mimics, eosinophilic fasciitis generally responds rapidly to glucocorticoid therapy. It is possible that unilateral eosinophilic fasciitis is under-recognized and can easily be misdiagnosed as another scleroderma variant if a full-thickness biopsy is not reviewed by a dermatopathologist. Recognition of this subtype of eosinophilic fasciitis is important given the profound differences in prognosis of eosinophilic fasciitis and other scleroderma variants.     

Necrotizing Fasciitis Due to Penicillin-resistant Streptococcus pneumoniae: Case Report and Review of the Literature

Necrotizing fasciitis (NF) is a life-threatening infection involving rapid necrosis of subcutaneous and fascial tissues. Streptococcus pneumoniae (SPN) soft tissue infection is exceedingly uncommon, reported primarily in patients with immunosuppression or other underlying conditions. We report a case of NF and septic shock in a healthy 32-year-old man, whose only predisposing factor was antecedent blunt trauma. Pathological examination and culture of the extensive tissue debridement were positive only for SPN. The serotype 9V isolate was penicillin (PCN)-resistant (MIC=2.0), and closely-related by pulse field gel electrophoresis and multilocus fingerprinting to clone France 9V-3, an important genetic reservoir for increasing PCN-resistance worldwide. This unique case has implications for our pathogenic under-standing and empiric management of NF.     

Pancreaticopleural Fistula: Report of Two Cases and Review of the Literature

Pancreaticopleural fistulas are a rare complication of pancreatitis. We report two cases from our institution and review 37 cases of pancreaticopleural fistulas identified in the literature. Endoscopic retrograde cholangiopancreatography was more sensitive compared to computed tomography in demonstrating pancreaticopleural fistulas (79% versus 43%, respectively). Medical therapy with total parenteral nutrition, octreotide, and/or chest tube placement was successful in resolving the pancreaticopleural fistula in up to 33% of cases. None of the patients who underwent pancreatic duct stent and/or nasopancreatic drain placement required surgical intervention. Endoscopic retrograde cholangiopancreatography is the initial test of choice when the diagnosis of pancreaticopleural fistula is suspected. Early endoscopic intervention with pancreatic duct stent placement is recommended given its high success rate in fistula closure. Medical therapies are useful adjuncts to endoscopic therapy, but rarely result in pancreaticopleural fistula closure alone. Surgical interventions should only be considered after failure of endoscopic and medical therapies.     

Severe Gastrointestinal Disorder Due to Capecitabine Associated with Dihydropyrimidine Dehydrogenase Deficiency: A Case Report and Literature Review

Dihydropyrimidine dehydrogenase (DPD) deficiency induces severe adverse events in patients receiving fluoropyrimidines. We encountered a 64-year-old DPD-deficient man with a severe capecitabine-related gastrointestinal disorder. He received capecitabine-containing chemotherapy after rectal cancer resection. During the first course of chemotherapy, he developed severe diarrhea, a fever, and hematochezia. Endoscopy revealed mucosal shedding with bleeding throughout the gastrointestinal tract. DPD deficiency was suspected because he developed many severe adverse events of capecitabine early and was finally confirmed based on the finding of a low DPD activity level in peripheral blood mononuclear cells. After one month of intensive care, hemostasis and mucosal healing were noted, although his gastrointestinal function did not improve, and he had persistent nutritional management issues.