Discrepancy In Diagnosis of Myelodysplastic Syndrome (MDS) Between Referral and Tertiary Care Centers: Experience at MD Anderson Cancer Center (MDACC)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1870-1870 ◽  
Author(s):  
Kiran Naqvi ◽  
Guillermo Garcia-Manero ◽  
Carlos E. Bueso-Ramos ◽  
Sherry Pierce ◽  
Tapan Kadia ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Median Survival ◽  
Treatment Plan ◽  
Tertiary Care ◽  
Classification Systems ◽  
Pathology Report ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Discordant Group ◽  
Tertiary Care Centers

Abstract Abstract 1870 Background: Patients (pts) referred to tertiary care centers occasionally have a final diagnosis that may differ from that made at the initial referring center. Previous studies have shown a discordance of 18% in the diagnoses of leukemia patients (AMJ. 1998;104:246-251). The aim of this study was to analyze the rate and implications of diagnostic discordance in pts with MDS referred to MDACC. Methods: A total of 915 pts presented to MDACC between September 2005 and December 2009 with an outside diagnoses of MDS. We reviewed the medical record of each patient for the pathology report on the diagnostic outside bone marrow slide sent for review. We also reviewed the outside hematopathology report sent with it. Diagnoses were coded according to WHO and the FAB MDS classification systems depending on time period. Information on pt characteristics including age, gender, IPSS, cytogenetics, complete blood count, % bone marrow blasts and transformation to acute myeloid leukemia (AML) were also recorded. Finally median survival was calculated using Kaplan-Meier method. Results: Out of the 915 charts reviewed, discordance in diagnosis was documented in 150 pts (16%). Sixty pts (40%) had an outside diagnoses of RA/RARS/RCMD/RCMD-RS (marrow blast <5%). Forty-six (77%) of these were diagnosed with RAEB (marrow blasts 5 to 20%) and 6 (10%) with RAEB-T (marrow blasts >20%) at MDACC. Similarly, 15 (26%) pts diagnosed with RAEB on the outside, were diagnosed with RA/RARS/RCMD/RCMD-RS at MDACC whereas 40 (70%) pts diagnosed with RAEB were diagnosed with RAEB-T. Only 2 pts diagnosed with RAEB-T on the outside were diagnosed with RAEB at MDACC. Moreover, 3 pts were diagnosed with chronic myelomonocytic leukemia (CMML), 3 with MDS unclassified, 2 with MDS/MPD had a different diagnoses at MDACC. The characteristics of pts with discordance were compared with the 765 pts without discordance. Essentially no difference was noted in the age, gender, cytogenetics, hemoglobin or platelet count between the two groups. A difference was noted with pts in the discordant group having a higher percent of RAEB-T (37% vs. 10%, p<0.0001). Similarly the discordant group was noted to have a greater number of high risk IPSS pts; 30% vs. 13% (p< 0.0001) and greater than 20% marrow blasts (25% vs. 8%, p<0.0001). No difference was noted in transformation to AML between the 2 groups: 13% vs. 8% (p=0.11). Seventy-eight (52%) pts in the discordant group and 372 (49%) pts in the non-discordant group died. Despite the differences noted above, no difference was observed in the median survival between the 2 groups by the Kaplan-Meier method (p=0.39). The median survival of both the groups was 15 months. Also only 19 pts in the discordant group were noted to have an outside report/marrow slide of > 6 months. Nine pts of these showed a higher marrow blast percentage than outside, indicating progression of disease. Conclusion: Discordance in the diagnosis of MDS pts is important as it can affect the treatment plan and overall prognosis of these patients. In our study, pts in the discordant group were observed to have a higher risk disease. However, no significant difference in survival was noted between the two groups. These results demonstrate the complexity of the morphological diagnosis of MDS and indicate that morphological classifications have a limited role in the prognosis of these pts. Analysis of implications of therapy based on diagnostic discordance is ongoing. Disclosures: No relevant conflicts of interest to declare.

scholarly journals IsHelicobacter pyloriBeing Treated Appropriately? A Study of Inpatients and Outpatients in a Tertiary Care Centre

2007 ◽  
Vol 21 (5) ◽  
pp. 285-288 ◽  
Author(s):  
Jose Nazareno ◽  
David K Driman ◽  
Paul Adams
Keyword(s):  
Tertiary Care ◽  
Gastric Biopsy ◽  
Pathology Report ◽  
Tertiary Care Centre ◽  
Delivery Of Care ◽  
Ulcer Disease ◽  
Appropriate Treatment ◽  
Significant Difference ◽  
H Pylori

BACKGROUND:Helicobacter pyloriis causally associated with peptic ulcer disease and gastric cancer. Although effective treatment is available, studies have shown that patients withH pyloriare often not well managed. Recently, there has also been increasing awareness of patient safety concerns arising from missed follow-up of abnormal test results.OBJECTIVE: To examine whether inpatients and outpatients diagnosed withH pylorireceive appropriate treatment.PATIENTS AND METHODS: All patients who were diagnosed withH pyloriby gastric biopsy in London, Ontario between January 1, 2004, and December 31, 2004, were identified. The hospital charts of these patients were reviewed. Outpatient office charts, clinic notes, pathology reports and endoscopy reports were also reviewed.RESULTS: One hundred ninety-three patients were diagnosed withH pyloriby gastric biopsy in 2004. Of the 193 patients, 143 (74%) were outpatients and 50 (26%) were inpatients. Overall, 89% of patients received treatment forH pylori. Ninety-two per cent of outpatients were treated, while only 60% of inpatients received treatment (P<0.001). Among the inpatients, the pathology report was available in 40% of the cases before the patient was discharged from the hospital. After discharge from the hospital, 30% of inpatients received appropriate treatment and follow-up. There was no significant difference in treatment whether the patient was admitted to a medical or a nonmedical service.CONCLUSION:H pyloriis treated relatively poorly in inpatients compared with outpatients. Results of the present study reveal opportunities to improve delivery of care for inpatients on a number of different levels. More research is needed to ensure safety, effectiveness and timeliness in the test result management process.

Treatment of Acute Myeloid Leukemia in a Community Hospital.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4575-4575
Author(s):  
Mandeep S. Dhami ◽  
Anca Bulgaru ◽  
Kandhasamy Jagathambal ◽  
Dinesh Kapur ◽  
Dennis E. Slater ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Myeloid Leukemia ◽  
Median Survival ◽  
Myeloid Leukemia ◽  
Community Hospital ◽  
Care Center ◽  
Tertiary Care ◽  
Cell Transplant ◽  
Number Of Patients ◽  
Acute Myeloid

Abstract Optimal management of patients with acute myeloid leukemia requires an accurate diagnosis along with cytogenetics and an intensive systemic chemotherapy regimen administered by a multidisciplinary team of experienced physicians, nurses and other support staff. It has been suggested that such complex patients should be treated only at tertiary care centers. However, it is often difficult for patients and families to receive care at teratiary care center which may be at a great distance from their home. Here we present a retrospective review of all patients diagnosed and treated for acute myeloid leukemia at William W Backus Hospital, a 213 bed acute care hospital serving a community of 70,000 in Norwich, Connecticut between the years 2000 and 2005. A total of 44 patients were treated during this period. There were 22 males and 22 females. The median age was 67.5 years. Bone Marrow samples were evaluated by a hematopathologist (histopathology, flowcytometry and cytogenetics) at a near-by tertiary care center. FAB subgroups and cytogenetics were similar to other published studies. APML patients are not included in this analysis. The median survival for the entire group was 14.7 months ranging from 2 days to 113 months. Fourteen patients were alive, all in continued clinical remission except one with relapsed disease and one patient remains transfusion dependent. Median survival was 15.2 months for men compared to 13.2 months for women. Four patients were referred for bone marrow/stem cell transplant after induction therapy. The limitation of this study is the relatively small number of patients as one would expect from a study done at a small community hospital. Nevertheless, it appears that the median survival of our patients is similar to a pooled analysis of five SWOG trials published by Gundacker et al. We conclude that most patients with acute myeloid leukemia can be managed in a community hospital with commitment and experience to treat such patients. Treatment outcomes (median survival) Study Number of Patients Under 55 55 – 65 65 – 75 Over 75 *Gundacker et al. Blood, 1 May 2006, volume 107, 3481–3485 Current study 44 17.1 m 18 m 11.7 m 5.7 m Gundacker et al* 968 18.8 m 9.0 m 6.9 m 3.5 m

Clinical Characteristics of T-Cell Lymphoma Associated Hemophagocytic Syndrome: Comparison of T-Cell Lymphoma with and without Hemophagocytic Syndrome.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3576-3576
Author(s):  
Hongyan Tong ◽  
Yanling Ren ◽  
Feng Xiao ◽  
Wenyuan Mai ◽  
Haitao Meng ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Median Survival ◽  
Clinical Characteristics ◽  
Hemophagocytic Syndrome ◽  
Cell Lymphoma ◽  
Initial Therapy ◽  
T Cell Lymphoma ◽  
Β2 Microglobulin ◽  
Significant Difference

Abstract T-cell lymphoma associated hemophagocytic syndrome (T-LAHS) has been regularly reported in Asia countries and is considered with extremely poor prognosis. The rate of definite diagnosis during early stage is low and the therapeutic outcome has been disappointed. We therefore compared T-cell lymphoma patients with and without hemophagocytic syndrome (HPS) in order to have a better understanding of the clinical characteristics of T-LAHS. One hundred and thirteen patients (66 men and 47 women, age from 12 to 80 years with the median age of 42) with aggressive T-cell lymphoma admitted to our department between January 2000 and December 2005 were included in this study, while 28 of them were with T-LAHS. The patients were divided into LAHS group and no-LAHS group. The clinical data including clinical manifestations and laboratory findings were compared between the two groups by using Chi-square test. The method of Kaplan and Meier was used to analyze overall survival (OS). The results showed that LAHS occured in about 1/4 of all the patients with T-cell lymphoma, which were all aggressive type. The elevated rates of lactate dehydrogenase (LDH) and ferritin were much higher in LAHS group than in no-LAHS group. β2-microglobulin and ovarian cancer antigen (CA125) were also elevated in both groups, but there was no significant difference. The rate of hypo-fibrinogen and liver dysfunction were higher in LAHS group than that in no-LAHS group. The rate of bone marrow infiltration in LAHS group is remarkably higher than that in no-LAHS group (57% vs 32%, p&lt;0.05). The median survival was 40 days (16 days - 22 months) in the LAHS group, and the median survival of 11 patients accepted chemotherapy more than 2 courses was 6 months. By contrast, the 2-year survival for no-LAHS group was 43%. There was significant difference between the two groups. Three patients undergoing plasmapheresis as initial therapy had survived for 3–6 months. These results indicate that high suspicion is required for early diagnosis of T-LAHS. In patients with fever, hepatosplenomegaly and cytopenia, simultaneously with serum markers such as LDH, ferritin, TG, CA125, and β2-microglobulin constantly increasing, T-LAHS should be considered. For patients without extranodal invasion or enlargement of lymph nodes, repeating biopsy of multiple sites of bone marrow may help improving the diagnosis rate. As for treatment, other more intensive regimens were not superior to CHOP regimen. While the overall outcome of treatment is still unsatisfied, plasmapheresis as initial therapy is worth considering.

Adequacy of Bone Marrow Trephine Biopsy in a Tertiary Care Center

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4702-4702 ◽  
Author(s):  
Emily K Rimmer ◽  
Donald S Houston ◽  
Kristine Roland
Keyword(s):  
Bone Marrow ◽  
Medical Students ◽  
Tertiary Care ◽  
Health Sciences ◽  
Bone Marrow Biopsies ◽  
Bone Marrow Trephine ◽  
Total Length ◽  
Significant Difference ◽  
Cancer Clinic

Abstract OBJECTIVES: To review the bone marrow trephine biopsies analyzed at the Health Sciences Centre in Winnipeg, Canada in 2007 and evaluate them in terms of published criteria for adequacy. METHODS: The Health Sciences Centre (HSC) is the largest tertiary care centre in the province of Manitoba, and houses the only leukemia treatment/bone marrow transplant ward. It also provides pathology services to the adjacent clinics of the provincial cancer agency. One thousand and twelve (1012) bone marrow aspirates and biopsies were identified from January 1 – December 31, 2007 through pathology records. Bone marrow biopsies performed on children (age &lt;18 years) and specimens referred from other centers were excluded. A total of 770 bone marrow aspirates and biopsies were identified as meeting the inclusion criteria and 67 were unavailable for evaluation. 703 biopsies were included in the final analysis. Data was collected on location of procedure (HSC ward vs. outpatient cancer clinic), operator and indication of biopsy. The total length of each biopsy and length of interpretable bone marrow were measured. The bone marrow biopsies were compared to published criteria for adequacy: 16 mm total length prior to processing (11 mm after processing), and 8 mm of interpretable marrow after processing. RESULTS: Using 8 mm of interpretable marrow as the criterion of adequacy, the overall adequacy rate was 67% (472/703). There was a significant difference in the percentage of adequate biopsies between operators. Hematologists obtained an adequacy rate of 81% (220/272), Registered Clinical Assistants (RCA) 63% (169/268), oncologists 56% (5/9), residents 50% (71/141), and medical students 54% (7/13), p&lt;0.001). The mean overall length of biopsy after processing was 14.5 mm (SD 5.3 mm), with 10 mm (SD 5.4 mm) of interpretable bone marrow. Hematologists obtained samples with a mean length of interpretable bone marrow of 12.4 mm (SD 5.8 mm), RCA 8.6 mm (SD 3.9 mm), oncologists 10 mm (SD 4.1 mm), residents 8.4 mm (SD 5.7 mm), and medical students 8.2 mm (SD 4.5 mm). There is a significant difference in length of core biopsies obtained by different operators. Hematologists get longer biopsies than residents (p≪0.01), RCA (p≪0.01) and medical students (p&lt;0.05). When looking specifically at those cases (n = 294) in which the requisition submitted at the time of the procedure stated an indication for which an adequate biopsy is crucial (detection of infiltration, diagnosis and staging of lymphoma) or when there was an inadequate/dry aspirate (n = 74), there was no significant difference in percentage of adequate biopsies when compared to the overall adequacy rate (70% vs. 67%, p=NS). When the biopsy was performed at the outpatient cancer clinic, the adequacy rate was 72.5% (342/472), whereas biopsies performed on HSC wards had an adequacy rate of 56.4% (133/236). CONCLUSIONS: A large proportion of bone marrow biopsies performed during the study period were of inadequate size. There was a significant difference in quality of bone marrow biopsies obtained by different operators, and among biopsies performed at different locations. The proportion of adequate samples was no better in cases where the aspirate was dry or where the suspected diagnosis should have mandated collection of an adequate sample. A multidimensional intervention including education and procedural changes will be implemented in order to improve the quality of bone marrow biopsies performed.

Analysis of Clinical Characterization of CMML.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4853-4853
Author(s):  
Suijing Wu ◽  
Xin Du ◽  
Wei Lin ◽  
Zilun Huang ◽  
Jianyu Weng ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Median Survival ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Prognostic Score ◽  
Monocyte Count ◽  
Average Survival ◽  
Significant Difference ◽  
L 62

Abstract Abstract 4853 Chronic myelomonocytic leukemia(CMML) was incorporated into the FBA classification of myelodysplastic syndromes(MDS) because dysplastic changes are commonly found in blood and bone marrow cells of patients with this order. However, sometimes doctors misdiagnose it because it may be distinguishable by only the presence of monocytosis(>1.0×109/L) in the blood.CMML is always a rather controversial type of MDS, some of patients present charaterication of myeloproliferation. In a further step, the WHO classification now includes CMML in a category of mixed myeloproliferative/myelodysplastic disorders, together with atypical CML and JMML,and proposed to separate CMML into CMML I and CMML II [Harris et al. Journal of Clinical Oncology,1999; Bennett International Journal of Hematology,2000]. Prognosis is also extremely variable in CMML.The median survival was about 19 months in 288 CMML patients included in Düsseldorf MDS Registry. There was no significant difference in median survival of MPD-CMML and MDS-CMML[Nosstinger et al. Leukemia Researsh.2001]. Germing reported the median of CMML patients who developed AML(18%) was 14 months,as compared to 20 months for patients who did not develop AML. [Germing et al. Leukemia and Lymphoma. 2004].In 2002, the M.D.Anderson Prognostic Score(MDAPS) using lymphocyte counts, hemoglobin level, medullary blast count, and presence of immature myeloid precursors in blood was developed by Onida[Onida et al. Blood. 2002]. Here we analyzed the clinical characterization of CMML in our hospital. 16 cases of CMML diagnosed according to the criteria of WHO classification were retrospectively analyzed. The median age was 51 years,and female/male was 11/5.Most of patients were median or older men with splenomegaly(37%), figure(30%), bone pain(25%), hepatomegaly(13%). White blood counts varyied from hypoleukocytosis to normal to hyperleukocytosis, median count was 27×109/L (1.3-74×109/L), median platelet count was 97×109/L(12-576×109/L),median hemoglobin count was 91g/l(62-138 g/l), median monocyte count was 4.6×109/L(2-14.5×109/L), median lymphocyte count was 4.4×109/L(1.0-33.8×109/L), LDH was elevated. Bone marrow was obviously active with dyshaematopoiesis, median blast cells of granulocyte were 8.5%(1%-18%), immature monocyte was 0-2.5%. 44% of patients had eosinophilic cells and/or basophil cells increase in bone marrow, and decrease in NAP, median score was 13(0-62). Immunophenotypic feature was CD13+CD14+CD56+,and FISH: BCR-ABL(-).The clinical course of CMML could remain stable for many years only low dose chemotherapy or without any treatment. On the other hand, there were patients with rapidly progressive disease. The Median survival was 20 months. CMML-I cases average survival was 50+ months, CMML-II was 18+ months≤ MDAPS showed low risk group had a average survival of 60+ months, intermediate group 1 and 2 was 30+ months and 17+ months,high risk patients with a average survival of only 1+ months. Only one case transformed into M4 as late as 3+ years after diagnosis of CMML(6%). In conclusion, the new criteria of WHO classification presents clinical feature of CMML better, and it can provide more effective prognostic parameters for risk assessment with MDAPS. Disclosures No relevant conflicts of interest to declare.

scholarly journals Prognostic Impact of Bone Marrow Erythropoiesis on Newly Diagnosed Plasma Cell Myeloma: A Single Institutional Analyses

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5579-5579
Author(s):  
Daniel E Ezekwudo ◽  
Rohit Singh ◽  
Bolanle Gbadamosi ◽  
Mark Micale ◽  
Ishmael Jaiyesimi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Clinical Outcome ◽  
Plasma Cell ◽  
Prognostic Impact ◽  
Plasma Cell Myeloma ◽  
Newly Diagnosed ◽  
Bone Marrow Biopsies ◽  
Kaplan Meier ◽  
Significant Difference ◽  
The Impact

Abstract Introduction: In plasma cell myeloma (PCM), tumor burden and activity plays an important role in diagnosis and prognosis (e.g. circulating plasma cells), however very little attention has been directed to the impact of the non-plasma cell component of the bone marrow. The presence of anemia has been used to distinguish PCM from smoldering myeloma; however this can be a non-specific finding as there are many potential causes of anemia besides PCM. We sought to determine if the level of erythropoiesis in bone marrow biopsies may be a more reliable prognostic factor. In the study herein, we assessed the level of bone marrow erythropoiesis in patients with newly diagnosed PCM, and compared those findings with cytogenetic results (CGs), other prognostic factors and overall clinical outcome. We hypothesized that patients with adequate erythropoiesis (AEp) are likely to have favorable cytogenetics and better outcome compared to those with decreased erythropoiesis (DEp). Methods: We retrospectively reviewed pathology database for bone marrow biopsies in patients with diagnosis of plasma cell myeloma (PCM) at Beaumont Hospital, an academic community center from 2012 and 2014. Biopsy cases without anemia were excluded. A total of 91 patients with plasma cell myeloma and anemia were identified. Each biopsy was re-examined to determine the level of erythropoiesis. The level of erythropoiesis was calculated by multiplying erythroid fraction (obtained from M:E ratio) with non-plasma cell bone marrow cellularity. Cases were separated into AEp and DEp using an erythroid compartment cut-off of 7.5% based on already established data. Kaplan-Meier analysis was used to compare survival between groups. Results: Demographic distribution of studied patients were 46 (50.1%) white, 39 (43%) African Americans and 6 (6.6%) others. Out of 91 cases analyzed, 38 (42%) had AEp whereas 53 (58%) had DEp. Among those with AEp, 23 (62%) had favorable CGs (defined as those without t (4, 14), t (14, 16), t (14, 20) or 17 p deletion); 15 (38%) had unfavorable CGs. Among those with DEp, 14 (26%) had favorable CGs whereas 39 (74%) had unfavorable cytogenetics. The vast majority of patients with favorable CGs were alive whether they had AEp (87%) or DEp (79%), thus CGs remained significant even after controlling for erythroid compartment (p = 0.03). Overall, those with AEp were noted to have significantly lower β-2 microglobulin (AEp median =2.42 mg/dL, DEp median = 4.50 mg/dL, p = 0.02). Kaplan-Meier analysis showed a significant difference in survival curves among the four groups (AEp with favorable CGs, AEp with unfavorable CGs, DEp with favorable CGs, DEp with unfavorable CGs, p<.0001). While the two groups with favorable CGs showed no significant difference (p=.6050), the two groups with unfavorable CGs did (p=.0027). Conclusion: Our findings suggest that patients with PCM and anemia are not a homogenous population. Assessment of the erythroid compartment in these patients reveals a population with AEp that has more favorable CGs and lower β-2 microglobulin than patients with DEp. Despite this finding, patients with favorable CGs had a favorable clinical outcome whether they had AEp or not, indicating that current therapies can overcome differences in erythropoiesis in that group. For patients with unfavorable CGs, however, those with AEp had superior survival outcome compared to those with DEp, indicating that there may be some prognostic or diagnostic utility to assessing erythropoiesis in patients who meet current criteria for PCM, and possibly, incorporating erythropoietic activity into diagnostic/prognostic schema. Disclosures No relevant conflicts of interest to declare.

Evaluation of the differences between extramedullary and bone marrow relapse in adult acute lymphoblastic leukemia patients in terms of clinical features and survival outcomes.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e19516-e19516
Author(s):  
Nilgun Sayınalp ◽  
Rafiye Ciftciler ◽  
Yahya Buyukasik ◽  
IC Haznedaroglu ◽  
Salih Aksu ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Lymphoblastic Leukemia ◽  
Clinical Features ◽  
Lymphoblastic Leukemia ◽  
Care Center ◽  
Tertiary Care ◽  
Survival Outcomes ◽  
Hematopoietic Stem ◽  
Significant Difference

e19516 Background: Acute lymphoblastic leukemia (ALL) in adult patients is an uncommon and difficult-to-treat hematological malignancy that is characterized by excess lymphoblasts in the bone marrow (BM). Although many patients achieve remission with chemotherapy, relapse rates are high and survival outcomes in adults are worse than pediatric patients. With uncontrolled proliferation and accumulation of these lymphoblasts, normal hematopoiesis is suppressed and infiltrates various extramedullary (EM) regions. The aim of this study is to evaluate the difference between EM and BM relapse in adult ALL patients in terms of clinical features and survival outcomes. Methods: In this study, we retrospectively analyzed 108 patients who were diagnosed as ALL and treated in our tertiary care center between 2003 and 2019. Statistical analyses were performed using the SPSS software version 25. Results: The study included 108 patients, consisting of 64 males and 44 females with a median age of 30 (range: 17-79 years). The majority of cases were B-cell in origin; 87 (80.6%) patients had B-ALL and 21 (19.4%) had T-ALL. Median follow-up duration for all patients was 21.1 months (range: 0.49-158.7 months). In the follow-up, 28 patients (25.9%) were received allogeneic hematopoietic stem cell transplantation. A total of 27 (25%) patients relapsed during the follow-up period. In 15 (13.9%) of 27 patients, only BM relapse was observed. EM relapse was observed in 12 (11.1%) patients. EM localizations were identified: brain [n = 2, 1.8%], lung [n = 1, 0.92%], retroperitoneum region [n = 1, 0.92%], kidney [n = 2, 1.8%], breast [n = 1, 0.92%], vertebral column [n = 3, 2.7%], spleen [n = 1, 0.92%], and uvea [n = 1, 0.92%]. All of the patients relapsed with bone marrow were B-ALL. Five of the patients (41.7%) with EM relapse were T-ALL (p = 0.006). No significant difference was observed in terms of gender (p = 0.16) and age (p = 0.12) in patients with BM relapse and EM relapse. Median overall survival (OS) was 42.3 months (95% CI: 15.6-69.0) for patients with BM relapse and 32.8 months (95% CI: 20.0-45.5) for patients with EM relapse (p = 0.42). Conclusions: In conclusion, EM relapse is common in ALL patients. We observed that EM relapse is more frequent, especially in patients with T-ALL cell origin. no significant difference was observed in both groups in terms of OS. ALL patients should be carefully followed up in terms of EM relapses as well as bone marrow relapse.

Gain of 1q as a Potential Adverse Prognostic Marker in Myelodysplastic Syndrome: Comparison with International Prognostic Scoring System Variables

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3640-3640 ◽  
Author(s):  
Hyun-Kyung Park ◽  
Dong Soon Lee ◽  
Hye Ryun Lee ◽  
Han Ik Cho ◽  
Hyun Kyung Kim ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Prognostic Marker ◽  
Median Survival ◽  
Survival Curve ◽  
International Prognostic Scoring System ◽  
Chromosomal Anomalies ◽  
Kaplan Meier ◽  
Meier Survival Curve ◽  
1Q Gain

Abstract The gain of the 1q region, which is a recurrent chromosomal aberration in B lymphoproliferative disorder, has been reported one of the most common anomalies in Korean myelodysplastic patients. Recently, risk based application of hypomethylating agents or tailored therapy in MDS rely on the prognostic variables of International Prognostic Scoring System (IPSS). To investigate the possibility of 1q gain as a new prognostic marker, we evaluated the prognostic impact of 1q gain, along with comparison with IPSS variables. A total of 117 patients with newly diagnosed MDS between 1997 and 2007 at the Seoul National University Hospital were investigated. Fluorescence in situ hybridization (FISH) studies with 5 specific probe(EGR1 for 5q31 deletion, D7S522 for 7q31 deletion, CEP8, D20S108 for 20q12 deletion, LSI 1p36/1q25 for 1q gain) and conventional G-banding karyotyping were performed on bone marrow aspirates. Other laboratory findings, such as hemoglobin(Hb), absolute neutrophil count(ANC), platelet count, bone marrow blast percent and IPSS score, and clinical data were collected through the individual medical records. The median age was 54 years and the male-to-female ratio was 1.4. Using WHO classification, refractory anemia(RA) was 27.4% and the other subgroups as follows: RA with ringed sideroblast(RARS), 3.4%; refractory cytopenia with multilineage dysplasia(RCMD), 8.5%; RCMD with ringed sideroblasts(RCMD-RS), 0.9%; RA with excess blasts-1(RAEB-1), 26.5%; RAEB-2, 31.6%; and 5q- syndrome, 1.7%. Cytogenetic abnormalities by FISH and G-banding were detected in 58 patients (49.6%). Most frequent anomaly was trisomy 8 occuring in 28 patients(23.9% of the 117 patients, 48.3% of the 58 patients with clonal cytogenetic abnormalities). Gain of 1q was the second common anomalies seen in 18 patients (15.4%) and other anomalies were −7/del7q (13.7%), −5/del5q (13.7%), and del20q (2.6%). G-banding showed gain of 1q in 7 cases, additional 11 patients with gain of 1q were revealed by FISH only. Patients with 1q gain showed a poor survival (median survival 23 months; n=18) compared to patients without 1q gain (median survival 60 months; p=0.02). EGR1 and D7S522 deletion by FISH also had a shorter median survival (8 months vs. 60 months p=0.0001, 16 months vs. 60 months p=0.005). The initial platelet count and blast count were found to affect overall survival, whereas CEP8 FISH, D20S108 FISH, Hb and ANC did not. Our results show that gain of 1q is associated with an adverse clinical outcome and can be considered as a poor cytogenetic risk factor of IPSS. In the Western study, the prevalence of 1q gain was low because most studies report G-banding result only. But it may be increased up to 2.5 fold higher by using FISH analysis in combination with G-banding. A gain of 1q could be a candidate as an adverse prognostic marker in clinical practice, which could help for risk-adapted therapies. Figure 1. Kaplan-Meier survival curve for chromosomal anomalies and IPSS. (A) gain of 1q. (B) −1/del(7q). (C) del(20q). Figure 1. Kaplan-Meier survival curve for chromosomal anomalies and IPSS. (A) gain of 1q. (B) −1/del(7q). (C) del(20q).

Combination Chemotherapy Leading In Advanced MDS Patients to a Rapid Clearence of Bone Marrow Blasts Prior Stem Cell transplantation (SCT) Is Superior to up-Front SCT Even with Intensified Conditioning for Long-Term Survival

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4020-4020
Author(s):  
Jaroslav Cermak ◽  
Antonin Vitek ◽  
Marketa Markova ◽  
Petr Cetkovsky
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Median Survival ◽  
Combination Chemotherapy ◽  
Significant Difference ◽  
Group A ◽  
Group B

Abstract Abstract 4020 A retrospective analysis of influence of different clinical and laboratory parameters on disease outcome was performed in a cohort of 43 patients with advanced myelodysplasia (MDS)(RAEB > 10% blasts + RAEB-T according to the FAB classification) who underwent allogeneic stem cell transplantation (SCT) in our institute within past 20 years; 21 patients were transplanted with < 10% of bone marrow (BM) blasts after 1 or 2 courses of induction followed by 1 or 2 courses of consolidation chemotherapy (Group A), 22 patients were transplanted with > 10% BM blasts either prior treated with combination chemotherapy or transplanted up-front with intensified conditioning (Group B). Median survival of all transplanted patients was 35,5 months (+/− 53,9 months) with a significant difference between Group A and B (57,5+/−62,3 months v.s. 18,0 +/−36,7 months, p=0.017). Estimated 3 year and 10 year survival for all patients were 53,5% and 41,9%, respectively. Estimated 3 and 10 year survival also significantly differed between Group A and B (71,4% and 57,1% for Group A and 36,4% and 27,3% for Group B). Complete remission (CR) rate was 44,2%, 18,6% patients relapsed (14,3% in Group A and 22,7% in Group B). No difference in overall survival was observed between patients with > 10% BM blasts transplanted either after chemotherapy or up-front (median survival: 26,8+/− 41,4 v.s. 18,0+/−33,8 months, respectively, p=0.65). Univariate analysis using Kaplan-Meier curves and log-rank2 test revealed as significant variables affecting overall survival: achievement of CR (p=0.007), achievement of < 10% BM blasts prior SCT (p=0.011), SCT performed < 4 months after diagnosis (p=0.031) and absence of relapse (p=0.046). Independent variables for determining overall survival (identified by Cox regression multivariate analysis) were: SCT performed < 4 months after dg. (p=0.003,χ2= 8,798), achievement of CR (p=0.011,χ2= 6,457), and age < 50 years (p=0.044,χ2= 4,053). None independent variable determining occurrence of relapse was found. Neither the percentage of BM blasts at the time of dg. and initial transfusion dependency, nor the donor origin (related or unrelated) and number of consolidation courses affected survival. Conclusions: combination chemotherapy leading to a rapid clearance of BM blasts below 10% followed by immediate SCT represented the best treatment option for younger patients with MDS with > 10% BM blasts. Patients transplanted with > 10% BM blasts at the time of conditioning had significantly inferior outcome either transplanted after previous chemotherapy or up-front with intensified conditioning. In this subset of patients, a possible benefit of addition of hypomethylating agents to the treatment schedules prior SCT should be studied. The study was supported by scientific programme MZCR 00023736. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Allogeneic bone marrow transplantation for childhood acute lymphoblastic leukemia in second remission after intensive primary and relapse therapy according to the BFM- and CoALL-protocols: results of the German Cooperative Study

Blood ◽  
1991 ◽  
Vol 78 (10) ◽  
pp. 2780-2784 ◽  
Author(s):  
R Dopfer ◽  
G Henze ◽  
C Bender-Gotze ◽  
W Ebell ◽  
G Ehninger ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Allogeneic Bone Marrow Transplantation ◽  
Allogeneic Bone ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Leukemic Relapse ◽  
Total Body ◽  
Relapse Therapy

Fifty-one children between 26 and 214 months of age (median, 100 months) with acute lymphoblastic leukemia (ALL) were grafted in second remission from HLA-identical sibling donors (except for two patients who were grafted with a marrow with 1 antigen-mismatch). Initial treatment and relapse therapy were similar in all patients according to the BFM- and CoALL-protocols (front line: 38 patients according to BFM- protocols and 13 patients according to CoALL-protocols; relapse: 12 patients in study ALL-REZ-BFM 83, 17 in ALL-REZ-BFM 85, 20 in ALL-REZ- BFM 87, and two in ALL-REZ-BFM 90). The conditioning regimens were different, consisting of cyclophosphamide (CY) total body irradiation (TBI) plus (n = 27), VP-16-TBI (n = 23), and CY-TBI and ARA-C (n = 1). Three patients had a second graft after conditioning with CY-TBI for the first transplantation. The second ablative regimen consisted of CY plus VP-16 in the first patient and CY plus busulfan in the two other patients, one of whom relapsed again. All patients but three had bone marrow (BM), either isolated or combined, relapses. Twenty-nine of the patients are in continuous complete remission (CCR), ranging from 1 to 67 months after transplantation with a median time of 30 months. One patient was lost to follow-up in continuous remission. Nine patients died from treatment-related complications (infections and graft-versus- host disease) and 12 patients suffered a leukemic relapse; three of them received a second graft and two are in CCR. Kaplan-Meier analysis yields an event-free survival (EFS) of 0.52 +/- 0.08. The probability of a 7-year relapse-free interval (RFI) is 0.68 +/- 0.08. EFS for patients with late relapses is 0.47 +/- 0.12 and for patients with early relapses 0.56 +/- 0.1. The RFI for patients with late relapses is 0.65 +/- 0.12 and for patients with early relapses 0.69 +/- 0.11. There is a nonsignificant trend towards superior results for patients grafted after conditioning with VP-16 plus TBI. When all patients who are not in CCR at day +125 (which is the median interval between relapse diagnosis and BM transplantation [BMT]) are excluded from the chemotherapy results, there is no significant difference between the results of BMT and chemotherapy for late relapses. On the other hand, there is a significant advantage between chemotherapy and BMT for early relapses over chemotherapy (P less than or equal to .01).

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