Early autoimmune gastritis presenting with a normal endoscopic appearance.

This report describes a patient with early-stage autoimmune gastritis (AIG) presenting with a normal endoscopic appearance. A 66-year-old man with autoimmune thyroiditis was suspected of having AIG because of a previous history of vitamin B12 deficiency when receiving steroid therapy for interstitial pneumonia 5 years earlier. At presentation, he tested positive for anti-parietal cell antibody (1:320) and anti-intrinsic factor antibody, but not for vitamin B12 deficiency. His gastrin level was elevated (338 pg/mL), but his pepsinogen (PG) I level (56.1 ng/mL) and PGI/PGII ratio (7.6) were normal. Endoscopically, neither atrophic nor inflammatory changes were observed. Histopathologic examination, however, showed mild atrophic changes with dense lymphocytic infiltration in the deep lamina propria and focal destruction of parietal cells in the greater curvature of the corpus. PGI-positive/MUC6-positive pseudo-pyloric metaplasia was observed in the area from which H+/K+-ATPase-positive parietal cells had disappeared. Chromogranin A immunostaining showed linear hyperplasia of enterochromaffin-like cells. By contrast, atrophic changes were not evident in the lesser curvature of the corpus, except for mild lymphocytic infiltration around and into the fundic glands. These serological and histopathological findings suggested that the patient had early-stage AIG with a normal endoscopic appearance.

Hyperhomocysteinemia-related lung disease and hemolytic anemia with bone marrow features masquerading as myelodysplasia.

Hyperhomocysteinemia is linked to TMA-related clinical symptoms such as apparent thromboembolism, microangiopathic hemolytic anemia (MAHA), and various types of end-organ damage due to microvascular thrombi; this is because high plasma levels of homocysteine impair the vascular endothelium. However, the association between hyperhomocysteinemia and pulmonary involvement is unclear. Here, we describe a 63-year-old male who was hospitalized with respiratory failure and MAHA with MDS-like features in the bone marrow. Plasma homocysteine levels were elevated significantly with 199.4 µmol/L (reference: 6.3-18.9) due to a homozygous (T/T) polymorphism for the 677C>T mutation within the MTHFR gene associated with chronic alcoholism-induced folate deficiency. Pulmonary lesions showed ground-glass opacity and there was pleural effusion. The patient was managed successfully with a combination of folate/mecobalamin supplementation, plasma exchange, and a methylprednisolone pulse, followed by oral prednisolone. Clinical symptoms, lung disease, MAHA, and bone marrow abnormalities improved as plasma homocysteine levels normalized.

Protein C Gene Mutation in an Older Adult Patient with Clostridium perfringens Septicemia-Related Visceral Vein Thrombosis.

A 78-year-old Japanese male with Clostridium perfringens septicemia and cholecystitis was found to have thrombosis in the left branch of intrahepatic portal vein as well as superior mesenteric vein. Visceral vein thrombosis (VVT) in this case was associated with protein C deficiency, due to a heterozygous mutation, p. Arg185Met. Our experience emphasizes that VVT, or other thromboembolic events, may occur in later life, triggered by environmental thrombosis risk factors, together with underlying hereditary protein C gene mutation.

Acquired Factor V Deficiency Associated with CFPM Administration.

BACKGROUND: Acquired factor V deficiency (AFVD) caused by Factor V (FV) inhibition is a rare event, characterized by prolonged prothrombin time and activated partial thromboplastin time. To date, various factors were reported as triggers for developing FV inhibitor. Clinical symptoms range from asymptomatic to life-threatening bleeding. Case Report and Conclusions: Here, we report an 84-year-old Japanese male on hemodialysis due to renal failure who developed subcutaneous hemorrhage after administration of cefepime (CFPM) to treat bacteremia. Deficient FV activity (< 1.0%) was identified and AFVD with FV inhibitor titer of 9 BU/mL was diagnosed. Although the patient had multiple risks for developing FV inhibitor, CFPM was thought to be the major culprit in this case. After the diagnosis, oral prednisolone (30 mg/day) was initiated, but the patient died of respiratory/cardiac failure, unrelated to AFVD.

Thrombotic Microangiopathy-Like Hemolysis in Vitamin B12 Deficiency-Related Macrocytic Anemia.

BACKGROUND: Hemolytic features in patients with pernicious anemia have not been emphasized. METHODS: Seven Japanese patients at 60 - 88 years of age with vitamin B12 deficiency-related hemolytic anemia were assessed. RESULTS: Serum vitamin B12 levels in these cases were 46 - 89 pg/mL (normal reference range: 233 - 914 pg/mL). Clinically, the patients presented with thrombotic microangiopathy (TMA)-like hemolytic features (including macrocytic anemia, schistocytes on blood smears, high serum lactate dehydrogenase, hyperbilirubinemia, and low serum haptoglobin). Six cases had type A gastritis (assessed by esophagogastroduodenoscopy with hypergastrinemia) with additional laboratory data of high plasma homocysteine levels and anti-intrinsic factor/anti-parietal cell antibodies. One case was in post-gastrectomy condition. Following treatment with vitamin B12, anemia resolved within 4 weeks in five of the seven cases except for two cases of delayed response. CONCLUSIONS: In elderly patients exhibiting hemolytic features in association with macrocytic anemia, vitamin B12 deficiency should be considered in the differential diagnosis.

[A case of disseminated carcinomatosis of bone marrow with disseminated intravascular coagulation caused by advanced colon cancer treated by mFOLFOX6].

A 45-year-old man was admitted to our hospital because of disseminated intravascular coagulation syndrome (DIC) and severe pain in his back and lumbar. Abdominal CT scan demonstrated lymph node enlargement in the whole body. FDG-PET revealed abnormal uptake of FDG in the thickening wall of the descending colon and the entire skeleton. Colonoscopy was performed continuously and revealed a poorly-differentiated adenocarcinoma of the descending colon. He was treated with the systemic chemotherapy of modified FOLFOX6 (mFOLFOX6). After one course of the treatment, DIC was resolved and severe back pain and lumbargo were improved.