This is the first study to use a standardized high-sensitivity FLAER-based flow cytometry assay and the recommended cutoff of 0.01% to identify cells with PNH phenotype in pediatric patients with ABMFD in the United States. The identification of a PNH population in 46% of ABMFD supports the recommendation for high sensitivity PNH testing in children with these disorders. As a less sensitive assay using a cutoff of ≥ 1% PNH population would have missed 10 (27%) patients with minor PNH population or rare cells with PNH phenotype. © 2017 International Clinical Cytometry Society.

Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare but highly undifferentiated, aggressive malignancy that primarily affects young women. Due to its early onset, unclear familial history and vague presenting symptoms, most SCCOHT patients present late with advanced disease. The prognosis is extremely poor, with <10% disease-free survival for advanced stages. Although several therapeutic regimens have been proposed, to date there is no consensus on the optimal strategy.Here, we describe a successful case of advanced-stage SCCOHT of the left ovary treated with cytoreductive surgery, semi-intense chemotherapy, high-dose consolidative chemotherapy, autologous hematopoietic stem cell transplantation and pelvic radiation with long-term survival. Given the almost universal mortality of advanced SCCOHT in long-term follow-up, we believe this case highlights the importance of prompt diagnosis when a young patient presents with abdominal swelling and hypercalcemia as well as early, aggressive, combined modality treatment. This case is also especially remarkable given the patient underwent fertility preservation surgery, which is not recommended by most of the current literature. However, as therapies improve and more young patients may survive SCCOHT, the question of fertility will increase in relevance. We believe the pros and cons of conservation should be discussed in detail with the patient.

Context.-Body fluids submitted to the hematology laboratory for cell counts may also be examined for the presence of malignancy. Previous studies evaluating the hematology laboratory's performance at detecting malignancy in body fluids have reached conflicting conclusions.Objective.-To investigate the hematology laboratory's ability to detect malignancy in body fluids by comparison with cytology.Design.-Retrospective analysis of 414 body fluid samples during an 18-month period, with introduction of new quality assurance measures after the first 210 cases. If no concurrent cytology was ordered, results were compared with recent previous and/or subsequent cytologic, histologic, or flow cytometric diagnoses.Results.-Of the initial 210 cases, the hematology laboratory detected 3 of 13 malignancies diagnosed by concurrent cytology (23% sensitivity), with no falsepositives (100% specificity). Malignancy was not identified on retrospective review of the hematology slides in the 10 discrepant cases. After the initial study, educational sessions on morphology for the medical technologists and a more thorough hematology-cytology correlation policy were implemented. The subsequent 204 hematology laboratory cases had increased sensitivity for the detection of malignancy (60%; 6 of 10). Definitive features of malignancy were seen in only one discrepant hematology laboratory slide on retrospective review. This case had not been flagged for hematopathologist review. None of the discrepancies before or after implementation of the additional quality assurance measures impacted patient care.Conclusions.-Body fluid processing by the hematology laboratory is not optimized for the detection of malignancy. Concurrent cytologic examination is critical for the detection of malignancy, and needs to be considered as cost-saving measures are increasingly implemented.(Arch Pathol Lab Med. 2014;138:651-657; doi: 10.5858/ arpa.2013-0295-OA) B ody fluid specimens are submitted to the hematology laboratory primarily for cell counting. In many cases, they are also examined for the presence of malignancy. The cytology laboratory likewise examines body fluids for the presence of malignancy, but not all body fluids are sent to both the hematology and cytology laboratories for evaluation.Cytology is able to diagnose 70% or more of metastatic carcinoma in malignant pleural effusions when cytologic smears and cell blocks are examined.1 The diagnostic yield of cytology is thought to depend on the skill of the pathologist and type of tumor, 2 with lower sensitivities seen in lymphomas (25%-50%), sarcomas (25%), and mesotheliomas (10%).1 Additional thoracenteses may improve the diagnostic yield.3 For ascites fluid, cytology is 40% to 65% sensitive at detecting malignancy, and sensitivity increases to more than 95% in carcinomatosis. 4 However, carcinomatosis accounts for only two-thirds of malignant peritoneal effusions.A perceived benefit of body fluid morphologic examination in the hematology laboratory is earlier reporting of negative results. Many h...