FDG PET/CT Maximum Tumor Dissemination to Predict Recurrence in Patients with Diffuse Large B‑Cell Lymphoma / 대한핵의학회잡지

Purpose@#We investigated the prognostic value of maximum tumor dissemination (Dmax), the distance between malignant lesions that were farthest apart, as assessed by fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT), and other clinical factors in patients with diffuse large B-cell lymphoma (DLBCL).We investigated the prognostic value of maximum tumor dissemination (Dmax), the distance between malignant lesions that were farthest apart, as assessed by fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT), and other clinical factors in patients with diffuse large B-cell lymphoma (DLBCL). @*Methods@#Patients who underwent FDG PET/CT for initial staging and treatment response evaluation of DLBCL were reviewed retrospectively. Baseline Dmax, maximum standardized uptake value, total summation of all metabolic tumor volumes (tMTV), and total summation of all total lesion glycolysis (tTLG) were measured. The treatment response was evaluated at the interim and end of first-line treatment (EOT) using the Deauville score (DS). FDG PET/CT parameters and other clinical factors including sex, age, serum lactate dehydrogenase (LDH) level, stage, performance status, and the International Prognostic Index (IPI) were analyzed to identify factors prognostic of the time to progression (TTP) and disease-specific survival (DSS). @*Results@#A total of 63 patients were included. Univariate survival analysis identified Dmax (> 275 mm), tMTV (> 180 mL), tTLG (> 1300), interim DS (≥ 4), and EOT DS (≥ 4) as significant predictors of poor TTP. Serum LDH level (> 640 IU/L), IPI (≥ 4), tMTV (> 180 mL), tTLG (> 1300), interim DS (≥ 4), and EOT DS (≥ 4) were significant predictors of DSS. After multivariate survival analysis, Dmax (P = 0.008) and EOT DS (P = 0.005) were independent predictors of TTP. EOT DS was an independent predictor of DSS (P = 0.029). @*Conclusions@#Dmax at the time of diagnosis and the EOT response assessed by FDG PET/CT provide useful prognostic information additive to the IPI in patients with DLBCL.

A Case of Peritonitis and Disseminated Mucormycosis Caused by Mucor circinelloides in a Patient with Nodal Marginal Zone B-cell Lymphoma

Mucormycosis is a fungal infection, which is difficult to treat due to its rapid dissemination and low susceptibility to anti-fungal agents. Peritonitis preceded by gastrointestinal mucormycosis is very rare, and only a few cases have been reported. We present a case of peritonitis and disseminated mucormycosis caused by Mucor circinelloides in an immunocompromised patient. A 59-year-old man, diagnosed with nodal marginal zone B-cell lymphoma, was diagnosed with liver failure due to severe septic shock. A white, woolly cotton-like growth, which was consistent with that of Mucor species, was isolated from ascites and sputum specimens. Targeted DNA sequencing confirmed the isolate as M. circinelloides with 100% identity. Despite anti-fungal treatment, the patient died after four days. This is a rare case of peritonitis and disseminated mucormycosis that was probably preceded by gastrointestinal mucormycosis caused by M. circinelloides, as determined by molecular methods. Accurate and rapid identification of mold using molecular methods might be necessary for early treatment in critical cases, and more cases should be clinically evaluated further.

Pleural Metastasis as Initial Presentation of Occult Gastric Cardia Cancer: A Possible Role of PET-CT in Diagnosis / Journal of the Korean Cancer Association, 대한암학회지

We report on a case of malignant pleural effusion as initial metastatic presentation of occult gastric cardia cancer in a young woman. To the best of our knowledge, this is the first report of gastric adenocarcinoma metastasized to pleura as an initial presentation. Location of cardia and signet ring cell histology may contribute to the manifestation. Utilization of positron emission tomography-computed tomography was helpful for proper diagnosis. For patients with such distinct clinical presentations, it would be appropriate to consider gastric cancer as one of the possible primary sites.

Loss of Red Cell A Antigen in a Patient with Acute Myeloid Leukemia: A Case Report and Review of the Literature / 대한수혈학회지

Red cell antigens, A, B, and H can be weakened or lost especially in patients with hematologic malignancies. We report a 42-year-old female patient with acute myeloid leukemia, who showed loss of A antigen on her red cells. She showed the persistence of leukemia in spite of three cycles of induction chemotherapy. Her ABO blood group showed a discrepancy: the cell type was O and the serum type was A. Adsorption/elution test could not identify the presence of A antigen on her red cells, and the test for A and B transferases was negative. ABO genotyping using PCR/restriction fragment length polymorphism and sequencing of exons 6 and 7 of the ABO gene demonstrated 467 C>T substitution in exon 7 and confirmed the genotype of A102/O01. She was transfused with leukapheresis products collected from donors with blood group A, but expired of severe sepsis. This is the first Korean case, in which red cell A antigen loss was genetically proven using sequencing, and underscores the necessity of ABO genotyping to solve the ABO discrepancy and to transfuse effectively.

Variant Burkitt-type translocation (8;22)(q24;q11) in plasma cell myeloma

Variant Burkitt-type translocation, t(8;22)(q24;q11), is very rare in plasma cell myeloma. We report a 51-year-old male patient with plasma cell myeloma, who showed t(8;22) (q24;q11). He suffered from pelvic pain for two months, and showed IgG, lambda type of monoclonal gammopathy (5.14 g/dL; 49.9% of protein). His bone marrow examination showed increased plasma cells (66.9% of all nucleated cells). Plasma cells (74.9% of all nucleated cells) and monoclonal spike (3.38 g/dL; 42.2%) persisted after three cycles of thalidomide and dexamethasone. Cytogenetic analysis showed complex chromosomal abnormalities: 44,XY,-1,t(2;5)(q33;q13),add(8)(q24.1),t(8;22)(q24.1;q11.2),add(10) (p15), der(11)t(1;11)(q21;p11.2),del(12)(p11.2p13),-13,-14,add(14)(q32),der(15)t(1;15)(p2 2;p11.2),-16,add(17)(q11.2),+21,+1-3mar[cp6]/46,XY[19]. To the best of our knowledge, this is the first report on plasma cell myeloma with a variant Burkitt-type t(8;22)(q24;q11) in the Korean patient. A review of 11 such cases in the literature, including the present case, implicated that plasma cell myeloma with t(8;22)(q24;q11) might be related to advanced stage and poor prognosis.

CD4+CD25highFoxP3+ Regulatory T-cells in Hematologic Diseases / 대한진단검사의학회지

BACKGROUND: CD4+CD25+ regulatory T-cells (Tregs) play a critical role in immune responses. We explored the status of Tregs in neoplastic and autoimmune hematologic diseases. We also evaluated the technical aspects of Treg measurement in terms of sample type and detection markers. METHODS: A total of 68 subjects were enrolled: 11 with AML, 8 with MDS, 10 with autoimmune diseases, and 39 controls. Tregs were analyzed in peripheral blood (PB) and bone marrow (BM) samples from each subject. Flow cytometry and the Human Regulatory T cell Staining Kit (eBioscience, USA) for CD4, CD25, and FoxP3 (forkhead box P3) were used. RESULTS: The CD4+CD25high/CD4 and CD4+CD25highFoxP3+/CD4 populations were significantly correlated (P<0.0001). The AML and high-risk MDS groups had significantly larger CD4+CD25high/CD4 and CD4+CD25highFoxP3+/CD4 populations in PB than the autoimmune (P=0.007 and 0.012, respectively) and control groups (P=0.004 and 0.006, respectively). Comparable findings were observed in BM. The CD4+CD25highFoxP3+/CD4 population was significantly larger in PB than in BM (P=0.0003). CONCLUSIONS: This study provides comparison data for Tregs in AML, MDS, and autoimmune hematologic diseases, and would be helpful for understanding the different immunologic bases of various hematologic diseases. Treg measurement using CD4, CD25, and/or FoxP3 in PB rather than in BM seems to be practical for routine hematologic purposes. Large-scale analysis of the diagnostic role of Treg measurement is needed.

Serum Prohepcidin Levels in Helicobacter Pylori Infected Patients with Iron Deficiency Anemia

BACKGROUND/AIMS: Helicobacter pylori (H. pylori) infection appears to subvert the human iron regulatory mechanism and thus upregulates hepcidin, resulting in unexplained iron-deficiency anemia (IDA). We evaluated serum prohepcidin levels before and after eradication of H. pylori in IDA patients to assess whether it plays a role in IDA related to H. pylori infection. METHODS: Subjects diagnosed with unexplained IDA underwent upper gastrointestinal endoscopy and colonoscopy to confirm H. pylori infection and to exclude gastrointestinal bleeding. Blood was sampled before treatment to eradicate H. pylori and again 1 month later. Serum prohepcidin levels were measured using a commercial enzyme-linked immunosorbent assay kit. RESULTS: Serum prohepcidin levels decreased significantly after oral iron replacement combined with H. pylori eradication (p = 0.011). The reduction ratio of serum prohepcidin levels after the treatment did not differ among the combined oral iron replacement and H. pylori eradication groups, the H. pylori eradication only group, and the iron replacement only group (p = 0.894). CONCLUSIONS: Serum prohepcidin levels decrease after both H. pylori eradication and oral iron administration, with improvement in IDA. Serum concentration of prohepcidin is related to the anemia status, rather than to the current status of H. pylori infection, in IDA patients.

A case of sideroblastic anemia caused by lead-containing herbal medication / 대한내과학회지

Although lead intoxication is commonly mentioned as a cause of sideroblastic anemia, no well-documented case exists in the literature. We encountered a patient with sideroblastic anemia caused by lead-containing herbal medicine. A 34-year-old woman was admitted to our hospital with abdominal pain. She had taken herbal medicine for her general health. Anemia, hyperbilirubinemia, and elevated lactic dehydrogenase were found from the laboratory data. Bone marrow biopsy showed pathological ringed sideroblasts. Her serum level of lead was high and the lead content of the tablet was higher than permitted. We diagnosed her with sideroblastic anemia secondary to lead poisoning caused by herbal medicine. We stopped her from taking herbal medicine and she gradually recovered from anemia.

The Clinical Guidelines for Myelodysplastic Syndrome

The myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis associated with multilineage cytopenias leading to serious morbidity or mortality, and the additional risk of leukemic transformation. The management of patients with MDS can be very complex and varies according to both the clinical manifestations in individual patients as well as the presence of complicating medical conditions. However, therapeutic dilemmas still exist for MDS due to the multifactorial pathogenetic features of the disease, its heterogeneous stages, and the elderly patient population. For these reasons, proper guidelines for management are necessary. This review describes the proper diagnosis for MDS, decision-making approaches for optimal therapeutic options that are based on a consideration of patient clinical factors and risk-based prognostic categories, and the use of recently available biospecific drugs such as hypomethylating agents that are potentially capable of abrogating the abnormalities associated with MDS. Proper indications and methods for transplantation, response criteria, management for iron overload for highly transfused patients and specific considerations for MDS in childhood are also described. All of these topics were discussed at the third symposium of AML/MDS working party on 3 March, 2007.

The Outcomes of Hypertransfusion in Major ABO Incompatible Allogeneic Stem Cell Transplantation

Major ABO incompatibility may be potentially associated with immediate or delayed hemolysis and delayed onset of erythropoiesis in patients receiving allogeneic hematopoietic stem cell transplantation (HSCT). To determine if hemolysis can be prevented by the inhibition of graft erythropoiesis, we performed hypertransfusion and assessed red cell transfusion requirement and independence. Between October 1995 and December 2001, 28 consecutive patients receiving major ABO incompatible HSCT at Samsung Medical Center were hypertransfused to maintain their hemoglobin levels at 15 g/dL or more. We retrospectively compared the outcomes of these patients with those of 47 patients at Asan Medical Center whose target hemoglobin levels were 10 g/dL. Reticulocyte engraftment was significantly delayed in hypertransfused group (51 days vs. 23 days; p= .001). There was no significant difference in the total amount of red cells transfused within 90 days post-HSCT (25 units vs. 26 units; p= .631). No significant difference in the time to red cell transfusion independence was observed between the two groups (63 days vs. 56 days; p=.165). In conclusion, we failed to improve red cell transfusion requirement and independence in major ABO incompatible HSCT with hypertransfusion.