Severe case of peripheral leukocytosis initially diagnosed as myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable, but possibly prefibrotic primary myelofibrosis.

Leukocytosis is occasionally seen in patients with presumptive but undiagnosed myeloproliferative disorders (MPD). A 74-year-old woman was admitted to our hospital for tarry stools, anemia, and marked peripheral leukocytosis of 1.4×10(5)/µL. Gastroenteroscopy revealed an acute gastric and duodenal mucosal lesion that was treated successfully via endoscopic hemoclipping. Bone marrow aspiration revealed marked megakaryocyte proliferation with atypia of naked nuclei and marrow hypercellularity (90% cellularity). A fluorescence in situ hybridization test could not detect the BCR-ABL fusion gene. Bone marrow aspiration later revealed further abnormalities of megakaryocytes. The patient died from cerebral bleeding. The present case fulfilled 2 of the 3 major criteria of primary myelofibrosis according to the World Health Organization 2008 classification:namely, megakaryocytic hyperplasia with hypercellular marrow and granulocytic hyperplasia. However, the megakaryocytic abnormality was not strictly compatible with the criteria. Instead, we considered prefibrotic primary myelofibrosis as a possibility, although myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable (MDS/MPN-U) was technically the correct diagnosis. The present case shows that MPN diagnosis remains difficult and suggests that other cases of peripheral leukocytosis with diagnosed MDS/MPN-U might include similar findings.

Cronkhite-Canada syndrome showing elevated levels of antinuclear and anticentromere antibody.

A 56-year-old female initially visited an otorhinolaryngologist because of an impaired sense of taste in September, 2010 and was referred to our facility in October, 2010. She was diagnosed with Basedow's disease for which she underwent subtotal thyroidectomy in 1984 and arthritis involving multiple joints, primarily affecting her hands. In addition, the anticentromere antibody (ACA) level was markedly high. On physical examination, alopecia as well as hyperpigmentation of the dorsum of the hands and back was observed. Dystrophic changes of the fingernails and a bilateral thumb abduction deformity were observed. Antinuclear antibodies were elevated. Gastrointestinal endoscopy and colonoscopy revealed the mucosa carpeted with strawberry-like polypoid lesions. Histopathological examination of the biopsied specimen of the stomach revealed a corkscrew-like appearance. Thus, the patient was diagnosed with Cronkhite-Canada syndrome (CCS). She admitted to our hospital in November, 2010. Oral prednisolone was administered with success. In July, 2012, her antimitochondrial M2 antibody level was elevated. To the best of our knowledge, the present case is the first patient with CCS, a history of Basedow's disease, and elevated levels of ACA and antimitochondrial M2 antibody. We consider the present case suggests CCS could be caused by immunological abnormality.

An Incarcerated Colon Inguinal Hernia That Perforated into the Scrotum and Exhibited an Air-Fluid Level.

There are few reports of a transverse colon inguinal hernia; furthermore, an inguinal hernia perforating the scrotum is rare. Here we report the case of a 79-year-old man who died after developing an incarcerated colon inguinal hernia that perforated the scrotum and exhibited an air-fluid level. The patient was referred to our hospital in November 2011 with a complaint of inability to move. Physical examination revealed an abnormally enlarged left scrotum and cold extremities. He reported a history of gastric cancer that was surgically treated more than 30 years ago. His white blood cell count and C-reactive protein level were elevated. Abdominal and inguinal computed tomography revealed that his transverse colon was incarcerated in the left inguinal canal. Free air and air-fluid level were observed around the transverse colon, suggestive of a perforation. The patient and his family refused any surgical intervention; therefore, he was treated with sultamicillin tosilate hydrate and cefotiam hydrochloride. However, he succumbed to panperitonitis 19 days after admission. The findings from this case indicate that the transverse colon can perforate into an inguinal hernia sac.

Two cases of acute erythroid leukemia presenting with marked macrocytic anemia, reticulocytosis and hemolysis.

Case 1. The laboratory findings of a hematological analysis of a 53-year-old woman with palpitations and dyspnea revealed the following: red blood cell (RBC) count: 9.4×10(5)/µL with 60.0‰ reticulocytes; Hb: 3.7 g/dL; mean corpuscular volume (MCV): 124.5 fL; white blood cell (WBC) count: 2,800/µL with 10.0% myeloblasts. Case 2. Similarly, a 42-year-old man with dizziness had a RBC count of 1.63×10(6)/µL with 24.0% reticulocytes, an Hb level of 6.0 g/dL, an MCV of 120.2 fL and a WBC count of 3,100/µL with 4.0% myeloblasts. Bone marrow aspirates in both patients confirmed a diagnosis of acute erythroid leukemia (AEL), which can present as marked macrocytic anemia with an MCV in excess of 120 fL and hemolysis.

Peripheral blood circulating immature cell counts predict CD34+ cell yields in G-CSF-induced PBPC mobilization in healthy donors.

BACKGROUND: It has been previously reported that the number of circulating immature cells (CIC) in peripheral blood (PB) estimates the number of CD34+ cells collected in G-CSF plus chemotherapy-induced PBPC mobilization. The correlation of CIC counts in PB with CD34+ cell yield and its usefulness was evaluated in G-CSF-induced PBPC mobilization for healthy donors. STUDY DESIGN AND METHODS: CIC counts in PB and CD34+ cell counts in the apheresis product from 122 collections were assessed, and the relationship between these two variables was evaluated with the Pearson rank correlation analysis, the chi-squared test, and the U-test. RESULTS: CIC counts were correlated weakly with the number of CD34+ cells per L of blood processed in the apheresis product (Pearson rank correlation analysis; r=0.357, p<0.0001). When a level of 1.7 x 10(9) CICs per L was selected as a cutoff value, the sensitivity and specificity for collecting more than 20 x 10(6) CD34+ cells per L of blood processed were 63.6 and 77.5 percent, respectively. CONCLUSION: The present study suggests that the number of CICs in PB may estimate the number of CD34+ cells collected. The data indicate that CIC counts above 1.7 x 10(9) per L can be used as a good predictor for PBPC collections containing more than 20 x 10(6) CD34+ cells per L of blood processed in a single apheresis procedure.

Predictive value of circulating immature cell counts in peripheral blood for timing of peripheral blood progenitor cell collection after G-CSF plus chemotherapy-induced mobilization.

BACKGROUND: Enumeration of CD34+ cells in peripheral blood (PB) before apheresis predicts the number of CD34+ cells collected, although flow cytometric techniques used are complex and expensive. In an attempt to determine the optimal timing for peripheral blood progenitor cell (PBPC) collection, the usefulness of circulating immature cell (CIC) counts in PB was evaluated. STUDY DESIGN AND METHODS: CIC counts in PB and CD34+ cell counts in the apheresis product from 249 collections were assessed, and the relationship between these two parameters was evaluated by with the Pearson rank correlation analysis, the Fisher exact test, and the U-test. RESULTS: CIC counts were correlated significantly with the number of CD34+ cells per kg of patient's body weight in the apheresis product (Pearson rank correlation analysis: r = 0.635, p < 0.0001). When a level of 1 x 10(9) CICs per L was selected as a cutoff value, the sensitivity and specificity for collecting more than 1 x 10(6) CD34+ cells per kg of body weight were 75.7 and 85.5 percent, respectively. CONCLUSION: The present study strongly suggests that the number of CICs in PB may estimate the number of CD34+ cells collected. The data indicate that CIC counts above 1 x 10(9) per L can be used as a good predictor for PBPC collections containing more than 1 x 10(6) CD34+ cells per kg of body weight in a single apheresis procedure.