Non-O blood group thrombotic thrombocytopenic purpura patients take longer to recover as measured by number of therapeutic plasma exchanges needed for platelet recovery.

INTRODUCTION: Therapeutic plasma exchange (TPE) is mainstay therapy for thrombotic thrombocytopenic purpura (TTP). However, it remains controversial if ABO type influences diagnosis or time to remission. MATERIALS AND METHODS: We investigated if ABO type influences length of TPE regimen in TTP patients with ADAMTS13 deficiency at our institution. Seventy out of 71 patients with suspected TTP who had ADAMTS13 activity measured were included. ADAMTS13 activity <10% defined those with idiopathic/acquired TTP (41/70). RESULTS: We found that among patients with ADAMTS13 deficiency, non-O patients required a significantly greater number of TPE (NoP) compared to O patients (p = 0.039). Additionally, patients with ADAMTS13 deficiency regardless of ABO type needed more TPE to achieve platelet recovery compared to those patients without deficiency (p = 0.00002). In regard to other variables that may affect response to therapy in TTP patients, we found no association between obesity and NoP; however, obesity rate was higher among ADAMTS13 deficient patients compared to overall obesity rate of our regional general population. Likewise, were found that blood group O did not occur with greater frequency in our cohort. CONCLUSIONS: Our data indicates that ABO may affect the NoP patients required for disease remission. We found that non-O patients needed more procedures to overcome their disease. Further work with greater number of patients will be needed to determine if specific non-O blood types require more procedures to recover their platelet count.

Absolute immature platelet counts in the setting of suspected heparin-induced thrombocytopenia may predict anti-PF4-heparin immunoassay testing results.

BACKGROUND: Heparin-induced-thrombocytopenia (HIT) is a disease mediated by antibodies to platelet factor 4 (PF4)-heparin complexes. Immature platelet fraction (%-IPF) and absolute immature platelet count (A-IPC) measure newly-released platelets into circulation and can prove useful in differentiating patients with thrombocytopenic presentations due to consumptive or hypoproduction processes. Therefore, we evaluated utility of A-IPC in a cohort of thrombocytopenic patients suspected of HIT. PATIENTS AND METHODS: Twenty-six thrombocytopenic patients (<150 × 109/L) tested for anti-PF4-heparin and 36 non-thrombocytopenic controls were included. Platelet count, %-IPF, and A-IPC were determined at time of anti-PF4-heparin testing. RESULTS: Sixteen patients tested anti-PF4-heparin negative and 10 tested positive. Patients with positive anti-PF4-heparin did not differ in A-IPC from normal range (7.2 ±â€¯2.9 × 109/L vs. 7.1 ±â€¯3.2 × 109/L respectively; p = 0.97). However, there was a significant A-IPC decrease in patients negative for anti-PF4-heparin compared to normal range and those testing anti-PF4-heparin positive (4.2 ±â€¯3.1 × 109/L vs. 7.1 ±â€¯3.2 × 109/L vs. 7.2 ±â€¯2.9 × 109/L respectively, p < 0.01). An A-IPC of greater than 5 × 109/L characterized 80% of anti-PF4-heparin positive cases. CONCLUSION: A-IPC measurements can complement anti-PF4-heparin testing of patients suspected of HIT while potentially predicting anti-PF4-heparin immunoassay results.

Comparison of GATA-3, mammaglobin, GCDFP-15 expression in breast carcinoma in serous effusions: A cell-block micro-array study.

BACKGROUND: GATA-3 is a potential marker for detection of metastatic breast carcinoma, reportedly more sensitive than mammaglobin (MAM) and GCDFP-15. We aim to compare the sensitivity of GATA-3, MAM and GCDFP-15 in determining the breast origin of malignant effusions. METHODS: Cell blocks from 27 cases of serous effusions positive for metastatic breast cancer were retrieved. Immunohistochemistry for GATA-3, MAM, gross cystic disease fluid protein 15 (GCDFP-15), estrogen receptor (ER) and progesterone receptor (PR) was performed on cell-block micro-array. Statistical analysis using two ways Chi square, one-way ANOVA and multiple regression was performed. RESULTS: The detection rate of breast cancer in serous fluid was significantly higher with GATA-3 (88.8 %, X2=15.9, p=0.00034) than with MAM (51.8 %) and GCDFP-15 (37.0 %). All ER positive cases (19) were GATA-3 positive. Conversely, all GATA-3 negative cases (3) were ER negative. The intensity of stain and percentage of positive cells were significantly higher with GATA-3 (p<0.0001) than with MAM and GCDFP-15. The intensity and percentage of positive cells score of GATA-3 were statistically associated with ER stain intensity and percentage of positive cell scores. CONCLUSIONS: GATA3 is a sensitive marker, superior to MAM and GCDFP-15 in determining the breast origin of metastatic adenocarcinoma. It is also strongly associated with ER expression.

Re-examination of 30-day survival and relapse rates in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.

BACKGROUND AND OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are characterized by microangiopathic hemolytic anemia and thrombocytopenia. Interestingly, markedly different survival rates have been reported despite increases in survivability. We studied TTP-HUS 30-day mortality and relapse rates of patients who received TPE at our institution and compared them to published data. PATIENTS AND METHODS: Retrospective study analyzed 30-day mortality and relapse rates attributed to TTP-HUS from 01/01/2008 to 12/31/2012 and compared them to comparable literature reporting mortality and survival. Studies describing other etiologies for TPE and different mortality time interval were excluded. RESULTS: Fifty-nine patients were analyzed and all were initially treated with TPE and corticosteroids. Eleven patients were classified as not having TTP-HUS due to testing or clinical reassessment which ruled in other etiologies, and 18/59 patients had ADAMTS13 activity <10%. Of remaining patients, 36/48 (75%) were diagnosed as idiopathic and 12/48 (25%) as secondary TTP-HUS. Patients received a mean of 12 TPEs (range 1-42); 42/48 (87.5%) patients had ADAMTS13 activity measured; complete response obtained in 39/48 (81.2%) patients (platelet count >100 x 10(9)/L); partial response in 4/48 (8%); and 5/48 (10.4%) did not have increases in platelet counts (2/5 of these patients died within the study period). Forty percent of patients obtained platelet counts >150 x 10(9)/L. Overall 30-day mortality for our patient cohort was 6.7% (4/59). Comparison of our mortality rate to combined data of five published studies of 16% (92/571) showed a significant difference, p = 0.04. Our relapse rate was 18.6% (11/59) similar to previous reports. CONCLUSIONS: Wide differences in mortality may be due to grouping of two distinct pathologic entities under TTP-HUS; and presence of confounding factors in the patient populations under study such as co-morbidities, promptness of TPE initiation, delay in diagnosis and therapeutic practice.