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Patients with chronic lymphocytic leukemia (CLL) may be more susceptible to Coronavirus disease 2019 (COVID-19) due to age, disease, and treatment-related immunosuppression. We aimed to assess risk factors of outcome and elucidate the impact of CLL-directed treatments on the course of COVID-19. We conducted a retrospective, international study, collectively including 941 patients with CLL and confirmed COVID-19. Data from the beginning of the pandemic until March 16, 2021, were collected from 91 centers. The risk factors of case fatality rate (CFR), disease severity, and overall survival (OS) were investigated. OS analysis was restricted to patients with severe COVID-19 (definition: hospitalization with need of oxygen or admission into an intensive care unit). CFR in patients with severe COVID-19 was 38.4%. OS was inferior for patients in all treatment categories compared to untreated (p

SUMMARY INTRODUCTION: EUS-guided gastroenterostomy (EUS-GE) is a novel procedure for palliation of malignant gastric outlet obstruction (GOO). Our aim was to evaluate the outcomes of this technique in our initial experience. METHODS: Patients with GOO from our institute were included. Technical success was defined as the successful creation of a gastroenterostomy. Clinical success was defined as the ability to tolerate a soft diet after the procedure. We assessed adverse events and diet tolerance 1 month after the procedure. RESULTS: Three patients were included. Technical and clinical success was achieved in all cases. There were no adverse events and good diet tolerance was observed 1 month after the procedure in the included patients. CONCLUSION: EUS-GE is a promising treatment for patients with GOO. RESUMO INTRODUÇÃO: A gastroenterostomia ecoguiada é um novo procedimento para paliação da obstrução maligna gastroduodenal. Nosso objetivo foi avaliar os resultados dessa técnica em nossa experiência inicial. MÉTODOS: Foram incluídos pacientes com obstrução maligna gastroduodenal de nossa instituição. O sucesso técnico foi definido como a realização adequada de uma gastroenterostomia. O sucesso clínico foi definido como boa aceitação de dieta pastosa durante a internação. Os eventos adversos e a aceitação alimentar foram avaliados um mês após o procedimento. RESULTADOS: Três pacientes foram incluídos. Os sucessos técnico e clínico foram alcançados em todos os casos. Não houve eventos adversos e a aceitação alimentar permaneceu adequada um mês após o procedimento nos pacientes incluídos. CONCLUSÃO: O EUS-GE é um tratamento promissor para pacientes com obstrução maligna gastroduodenal.

BackgroundAcquired hemophilia A is a rare autoimmune disease with clinically often significant bleeding diathesis resulting from circulating autoantibodies inhibiting coagulation factor VIII. Half of acquired hemophilia A cases are associated with an underlying disorder, such as autoimmune diseases, cancer, or use of certain drugs, or occur during pregnancy and in the postpartum period. In the other half, no underlying cause is identified. An association of acquired hemophilia A with plasma cell neoplasm seems to be extremely rare.Case presentationWe describe a case of a 77-year-old Swiss Caucasian man who was diagnosed with acquired hemophilia A and smoldering multiple myeloma as an underlying cause. Acquired hemophilia A was treated with prednisolone, cyclophosphamide, and immunoadsorption. Extensive workup revealed a plasma cell neoplasm as the only disorder associated with or underlying the acquired hemophilia A. For long-term control of acquired hemophilia A, we considered treatment of the plasma cell neoplasm necessary, and a VRD (bortezomib, lenalidomide, and dexamethasone) regimen was initiated. Due to multiple complications, VRD was reduced to VRD-lite after two cycles. After nine cycles of induction therapy and five cycles of consolidation therapy, the patient is in complete remission of his acquired hemophilia A and very good partial remission of the plasma cell neoplasm. We conducted a literature review to identify additional cases of this rare association and identified 15 other cases. Case descriptions, including the sequence of occurrence of acquired hemophilia A and plasma cell neoplasm , treatment, evolution, and outcome are presented.Discussion and conclusionsOur case, together with 15 other cases described in the literature, underscore the possibility of plasma cell neoplasm as an underlying cause of acquired hemophilia A. Physicians should consider including protein electrophoresis, immunofixation, and analysis of free light chains in laboratory diagnostics when treating a patient with acquired hemophilia A. The occurrence of excessive and unexplained bleeding in patients diagnosed with plasma cell neoplasm should raise suspicion of secondary acquired hemophilia A and trigger the request for coagulation tests, particularly in patients treated with immunomodulatory drugs such as thalidomide or lenalidomide. Additionally, early intervention with immunoadsorption can be lifesaving in cases with high-titer factor VIII inhibitors, especially when surgical interventions are necessary.

The use of ibrutinib is hampered by major bleeding events and atrial fibrillation. Speculating whether randomized controlled trials might underestimate the risk of adverse events in clinical practice, we conducted a systematic review and meta-analysis studying patients treated in any setting and indication. We systematically searched the literature using MEDLINE and EMBASE databases for case series, cohort studies, or randomized controlled trials and retrieved all data in parallel. Proportions of patients with adverse events were pooled in relevant subgroups using the binominal distribution and Freeman-Tukey double arcsine transformation. Among 2'537 records screened, 85 were finally included, comprising 7'317 patients. Methodological quality according to the Newcastle-Ottawa Scale was rated as moderate to poor with regard to bleeding events and atrial fibrillation; 106 studies were excluded because of missing data at all. Reported events varied substantially between 0 % and 78 % (any bleedings), 0 % and 25 % (major bleedings), and 0 % and 38 % (new-onset atrial fibrillation). Pooled estimates were 28 % (95 % confidence interval 22 %, 34 %), 3 % (2 %, 4 %), and 8 % respectively (7 %, 10 %). The risk of events was higher in studies with an older population, high ibrutinib dosage, thrombocytopenia, antithrombotic treatment, and retrospective studies. In conclusions, reporting of bleeding events and atrial fibrillation varied substantially among studies. These observations, in combination with the estimates obtained, suggest a relevant risk in clinical practice.

Chronic lymphocytic leukemia (CLL) is a disease of the elderly, characterized by immunodeficiency. Hence, patients with CLL might be considered more susceptible to severe complications from COVID-19. We undertook this retrospective international multicenter study to characterize the course of COVID-19 in patients with CLL and identify potential predictors of outcome. Of 190 patients with CLL and confirmed COVID-19 diagnosed between 28/03/2020 and 22/05/2020, 151 (79%) presented with severe COVID-19 (need of oxygen and/or intensive care admission). Severe COVID-19 was associated with more advanced age (≥65 years) (odds ratio 3.72 [95% CI 1.79–7.71]). Only 60 patients (39.7%) with severe COVID-19 were receiving or had recent (≤12 months) treatment for CLL at the time of COVID-19 versus 30/39 (76.9%) patients with mild disease. Hospitalization rate for severe COVID-19 was lower (p < 0.05) for patients on ibrutinib versus those on other regimens or off treatment. Of 151 patients with severe disease, 55 (36.4%) succumbed versus only 1/38 (2.6%) with mild disease; age and comorbidities did not impact on mortality. In CLL, (1) COVID-19 severity increases with age; (2) antileukemic treatment (particularly BTK inhibitors) appears to exert a protective effect; (3) age and comorbidities did not impact on mortality, alluding to a relevant role of CLL and immunodeficiency.

Hairy cell leukemia (HCL) remains an incurable disease. However, first-line treatment with either intravenous or subcutaneous cladribine generally leads to long-lasting remissions. Although there are excellent long-term data for intravenous application, similar data regarding subcutaneous administration are lacking. We therefore analyzed the long-term outcome of 3 prospective multicenter clinical trials on subcutaneous cladribine performed by the Swiss Group for Clinical Cancer Research (SAKK), which recruited 221 patients with classical HCL between 1993 and 2005. Median overall survival from start of treatment was not reached. Pretreatment anemia, higher Eastern Cooperative Oncology Group score, and higher age were associated with poorer overall survival in multivariable analysis, whereas early progression at 24 and 36 months had no significant impact on overall survival. Second-line treatment was necessary in 53 (23.7%) patients after a median of 5 (range, 0.2-20.4) years, and first retreatment was mainly monotherapy with cladribine (66%) or rituximab (15.1%) or a combination of these drugs (15.1%). A total of 44 (19.9%) patients developed second primary malignancies with a median time to occurrence of 5.7 (range, 0.01-17.5) years. Second primary malignancies were the main cause for death (14; 27.5%). Compared with a matched normal Swiss population, the incidence of second primary malignancies was not increased. However, survival of patients with HCL was slightly inferior by comparison (P = .036). In conclusion, the outcome of HCL patients treated with subcutaneous cladribine is excellent, and in most patients, 1 cycle of subcutaneous cladribine is sufficient for long-term disease control.

Our aim in this study was to compare the efficiency of 25G versus 22G needles in diagnosing solid pancreatic lesions by EUS-FNA. We performed a systematic review and meta-analysis. Studies were identified in five databases using an extensive search strategy. Only randomized trials comparing 22G and 25G needles were included. The results were analyzed by fixed and random effects. A total of 504 studies were found in the search, among which 4 randomized studies were selected for inclusion in the analysis. A total of 462 patients were evaluated (233: 25G needle/229: 22G needle). The diagnostic sensitivity was 93% for the 25G needle and 91% for the 22G needle. The specificity of the 25G needle was 87%, and that of the 22G needle was 83%. The positive likelihood ratio was 4.57 for the 25G needle and 4.26 for the 22G needle. The area under the sROC curve for the 25G needle was 0.9705, and it was 0.9795 for the 22G needle, with no statistically significant difference between them (p=0.497). Based on randomized studies, this meta-analysis did not demonstrate a significant difference between the 22G and 25G needles used during EUS-FNA in the diagnosis of solid pancreatic lesions.

Practical guidance on diagnostic and prognostic use of next generation sequencing (NGS) in multiple myeloma (MM) and other plasma cell neoplasms (PCN) has yet to be developed. To this end, we analyzed the correlation of NGS data with the degree of bone marrow (BM) plasma cell (PC) involvement obtained by cytomorphology (CM), histopathology (HP), and multiparameter flow cytometry (MFC) from patients with PCN/MM. To perform this correlation, we analyzed 90 PCN cases, that included MM (n=77), MGUS (n=7), AL-amyloidosis (ALA) (n=4) and solitary plasmocytoma (SP) (n=2). The degree of BM infiltration (percent of PC) was defined by MFC as grade I 3%; by CM as grade I 30% and by HP as grade I 30%. The newly designed NGS panel consisted of 15 genes including splice sites or hotspots: BRAF (exons 11, 15), CCND1, DIS3, EGR1, TENT5C (FAM46C), FGFR3, IDH1 (exon 4), IDH2 (exon 4), IRF4 (exon 3), KRAS (exons 2, 3), MYD88 (L265P), NRAS (exons 2, 3) PRDM1, TP53 and TRAF3. Genes and hotspots were chosen according to the frequency in the literature, prognostic impact, and possible function as therapeutic targets (Chapman et al., Nature 2011; Walker et al., JCO 2015). PC enrichment based on CD138+ magnetic cell sorting of marrow samples was successfully performed before the NGS procedure in all cases. In total, 102 mutations were detected by NGS in 64/90 (71%) cases analysed, 26 cases (29%) showed no mutations. In 41 cases (45%) one mutation/sample was detected and in 23 (26%) two to five mutations/sample. The proportion of cases affected by mutations was 59/77 patients (77%) in MM, 2/7 (29%) in MGUS and 1/4 (25%) in ALA. The most frequent mutations across all PCNs types were KRAS 22/90 (24%) and NRAS 14/90 (16%), followed by DIS3 and TENT5C in 11/90 cases each or 12% (Table 1). Mutational load correlated with degree of bone marrow infiltration: BM samples with no mutation had the lowest plasma cell infiltration: mean 1.6% by MFC (range, 0.003 - 11.7%), 20% by CM (1 - 90%), and 24% by HP (1 - 80%). Presence of at least one mutation/sample corresponded to a higher degree of BM involvement with a mean of 11% pathologic PC by MFC (range, 0.002 - 62%), and ~50% (3 - 100%) as defined by both CM and HP (Figure 1). The majority of samples without mutation (26 cases, 29%) were obtained from patients with initial BM evaluation for PCN suspicion (19/26 cases or 73% of this subgroup), whereas BM samples with >1 mutation (23 cases, 26%) were mostly acquired from patients with relapsed/refractory disease (15/23 cases, or 65% of this subgroup). TP53 mutated cases had the highest percentage of PC infiltration by all methods and were mostly correlated to relapsed/refractory MM (rrMM). In conclusion, the probability to detect a mutation by NGS in the BM was highest in samples with >10% clonal PC by flow cytometry, or >20% PC by CM/HP. We propose further evaluation of these thresholds as a practical cut-off for processing of samples by NGS at initial PCN/MM diagnosis, whereas disease progression is commonly associated to higher rates of mutations. TP53 mutation was detected in all but one samples from rrMM and was associated with the highest degree of BM involvement. Further large-scale studies are warranted. Disclosures No relevant conflicts of interest to declare.

Background Worth syndrome or autosomal dominant endosteal hyperostosis (ADEH) is an extremely rare genetic disease involving increased bone density. To the author's knowledge, this is the second case report of a family with neurologic involvement associated with this condition along with its surgical treatment. The most effective treatment for clinically significant neurologic symptoms in this scenario is currently unknown, and there is sparse experience on surgical treatment for this condition reported in the literature. Therefore we aim to make a contribution to the identification of a standard and consistently successful surgical management. Case Description Two patients, mother (Patient 1) and daughter (Patient 2), were diagnosed with Worth syndrome. Both presented with the typical facial characteristics described for ADEH. Interestingly, Patient 1 presented the novel mutation in the LRP5 gene that is associated with different conditions involving increased bone density. Although neurologic symptoms are infrequent in ADEH, both referred chronic headache, nausea, and vomiting. Neuroimaging showed an increased cranial bone density and Chiari I malformation. The patients underwent a midline suboccipital craniectomy with excision of the posterior arch of C1 and duroplasty. However, due to a symptomatic recurrence 5 years after surgery, Patient 1 was reoperated on. We extended the craniectomy and also carried out a C2 laminectomy. Conclusion After surgical interventions, patients' neurologic symptoms were successfully resolved. This report shows that posterior fossa decompression including duroplasty may be a valid treatment option in case of neurologic involvement.

Background Treatment of multiple myeloma has changed considerably over the last two decades with remarkable reduction in mortality rates in clinical trials and in population-based studies. Since health care systems and patient management differ between countries, population-based data from cancer registries with high coverage may provide further insight into real-life achievements and unmet needs. We report on the first population-based nation-wide study of incidence, mortality and survival of multiple myeloma in Switzerland covering the era of autologous stem cell transplantation and the first proteasome inhibitors and immunomodulatory drugs. Methods We performed a retrospective registry study with data from the National Institute for Cancer Epidemiology and Registration (NICER) database in Switzerland from 1994 to 2013. Results We identified 5770 patients with multiple myeloma. Incidence has increased from 419 new cases per year in 1994–1998 to 557 new cases per year in 2009–2013 while the age-adjusted incidence rate remained stable at 4.7–5.0 per 100′000 person-years. Five- and 10-year relative survival increased from 32.6% (95%CI 29.3–36.0) and 17.8% (95%CI 14.9–21.0) in 1994–1998 to 46.4% (95%CI 43.3–49.3) and 25.0% (95%CI 21.9–28.3) in 2009–2013. Conclusion The increase in incidence can be attributed to demographic changes. There is a trend to longer relative survival in all age groups with substantial increase in myeloma patients aged less than 75 years and only minimal changes in older persons.

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by circulating autoantibodies inhibiting coagulation factor VIII (FVIII), leading to a clinically significant bleeding diathesis. While in half of AHA cases, no underlying cause is identified, in the other half an association with autoimmune diseases, cancer, the use of certain drugs, pregnancy or the post-partum period is found. We present a case with severe bleeding in which AHA was diagnosed and subsequently a smoldering myeloma was found as underlying disease (case 16, Table 1). We conducted a systematic review of the literature in PubMed looking for the association of AHA and plasma cell neoplasm (PCN) (key words used: hemophilia, inhibitor, factor VIII, myeloma, plasma cell disorder or neoplasms, smoldering myeloma, MGUS, monoclonal gammopathy, paraprotein) and identified 15 additional cases. Description of the cases, sequence of occurrence of both diseases, their treatment, evolution and outcome are here analyzed (Table 1). We found that patients having AHA and PCN were more often males (9/16, 56%), had a median age of 61.5 (range 43-87) years at AHA diagnosis. The most common pattern of bleeding was a soft tissue bleeding. In two cases, AHA was diagnosed after excessive post-operative bleeding, in one patient following a life threatening pericardial bleeding and a hemarthrosis. AHA with active bleeding was the presenting sign in 6 cases (38%) (Table 1, cases 4, 5, 6, 10, 13, 16), in search for an underlying diseases, a PCN was diagnosed subsequently. In the other 10 cases, AHA was diagnosed after PCN. In 3 of them, the occurrence of AHA was interpreted to be secondary to multiple myeloma treatment; the implicated drugs were interferon alpha in one and lenalidomide in two cases. Information about the type of monoclonal gammopathy was available in 11 cases; no particular type of paraprotein or clonal light-chain was discernable. Factor concentrates were used in 12 cases to treat relevant bleedings. Further treatment of AHA consisted of immunosuppression with steroids (N=4), steroids in combination with cyclophosphamide (N=5), or cyclophosphamide alone (N=1). Rituximab was used in 3 cases. Plasma exchange to remove FVIII antibodies was performed in 2 cases, and immunoadsorption was done in our patient. Different therapy regimens were used to treat the underlying PCN over the years (Table 1). At the time of reporting 13 patients were alive, while 2 of 3 patients who died, died of bleeding complications. Information on outcome of AHA and/or PCN was available for 9 of 13 survivors: 6 had normal coagulation tests, while FVIII activity was mildly reduced in two. PCN was in complete and partial remission in 7 and 2 cases, respectively (table 1). Management of AHA is based on four pillars: avoidance of procedures that may have induced bleedings, control of bleeding, inhibitor eradication and treatment of the underlying disease. Our case, together with those described in the literature, emphasizes the possibility of PCN as an underlying cause of AHA. Accordingly, diagnostics including protein-electrophoresis, immune-fixation and free light chains should be considered in patients with AHA. In our experience, early intervention with immunoadsorption can be lifesaving in cases with high FVIII inhibitor titers. The occurrence of excessive unexplained bleeding in PCN should raise the suspicion of secondary AHA and trigger the investigation of coagulations tests. Whether PCN treatment alone can control AHA in these cases remains open, 11/16 (69%) of the reported cases received treatment for both diseases. Disclosures Jalowiec: Amgen: Other: Travel grant; Pfizer: Other: Travel grant; Novartis: Other: Travel grant. Andres:AbbVie: Membership on an entity's Board of Directors or advisory committees; Janssen-Cilag: Membership on an entity's Board of Directors or advisory committees; Roche: Other: Travel support ; Gilead: Other: Travel support ; Celgene: Membership on an entity's Board of Directors or advisory committees; Novartis: Other: Travel support ; Mundipharma: Other: Travel support ; AbbVie: Other: Travel support; Celgene: Other: Travel support . Kremer Hovinga:CSL-Behring: Honoraria, Other: Honoraria go to employer (Insel Gruppe AG, Department of Hematology); Ablynx/Sanofi: Consultancy, Honoraria, Other: Honoraria go to employer (Insel Gruppe AG, Department of Hematology); Roche: Honoraria, Other: Honoraria go to employer (Insel Gruppe AG, Department of Hematology); Shire: Consultancy, Other: Honoraria go to employer (Insel Gruppe AG, Department of Hematology), Research Funding; Siemens: Honoraria, Other: Honoraria go to employer (Insel Gruppe AG, Department of Hematology).

Introduction : Hairy cell leukemia (HCL) is a chronic mature B-cell neoplasm with a very indolent clinical course and patients may survive for many decades. First-line treatment with purine analogues such as cladribine (Cld) is considered standard of care since it is very efficient and induces profound remissions. However, patients with HCL often relapse after purine analogues and repeated treatment may increase morbidity and mortality. Despite good clinical evidence of long term control of the disease by several mainly single center studies of patients treated with purine analogues, there is only one study analyzing mainly subcutaneous (sc) treated patients based on registry data. We therefore performed a pooled long-term follow-up analysis of our prospective multicenter studies treating patients with sc Cld focusing on survival, secondary malignancy and retreatment. Materials and Methods : The SAKK included patients treated for HCL in 4 studies between 1993 and 2005. Three studies focused on first-line regimens with sc Cld, whereas the fourth protocol focused on the effect of Rituximab monotherapy in patients pretreated with Cld. Classical morphologic, immunohistochemistry and flow cytometry criteria were used as inclusion criteria and response was assessed by established criteria. Treatment algorithms in the 4 studies were as follows: 1) 5 days of Cld 0.14mg/kg sc followed by max 2 cycles of 7 days of Cld 0.1mg/kg sc in case of minor response or no response (SAKK 32/93); 2) Single shot of Cld 0.25mg/kg sc followed by a maximum of 2 cycles of 0.14mg/kg sc for 5 day in case of minor response, no response or relapse (SAKK 32/95); 3) 5 consecutive days of Cld 0.14mg/kg sc versus the same dose in 5 weekly applications (SAKK 32/98); 4) Rituximab 375 mg/m2iv weekly for 4 weeks in relapsed patients (SAKK 31/98). SAKK 32/93 included 63, SAKK 32/95 74 and SAKK 32/98 100 patients. Of the 26 patients registered in 31/98 20 were already in SAKK 32/93, 32/95 and 32/98. Therefore, we also included the treatment information and follow-up data of these 20 patients. All patients were subject to life-long follow-up within the clinical trials. Further information including secondary malignancies and retreatments were obtained by sending out questionnaires to the treating physicians of the study patients. Of the 237 patients 4 patients were in two of the studies and 10 patients have been excluded because of non-classical HCL phenotype. Therefore, a total of 223 patients were included in the analysis. Overall survival and follow-up time were assessed by Kaplan-Meier and reverse Kaplan-Meier method, respectively. Results : The median age of patients at the time of diagnosis was 55 (range 21 to 96) years, 50 patients were female (22.4%) and 173 (77.6%) male. At the time of data analysis, the median follow-up time was 12.1 (95%-CI 10.0 to 14.0) years. A total of 129 (57.8%) patients had the last follow-up information more than two years prior to the data cut-off in May 2016, however, the available information of all patients was used for the sub-analyses including secondary malignancies or retreatment. By the cut-off date, 49 patients have died, 14 (28.6%) due to secondary malignancies and 7 (14.3%) due to HCL progression. Median overall survival from diagnosis was 31.6 (95%-CI 31.6 to 37.8) years. Retreatment was necessary in 53 (23.7%) patients after a mean of 6 (0.2 to 20.4) years and first retreatment was mainly Cld (64%), rituximab (19%) or Cld and rituximab (13%). 21 patients (9.4%) required more than one retreatment with a mean number of 1.57 (range 1 to 5) treatments. A total of 42 (18.8%) patients developed secondary malignancies with an average time to occurrence of 7.1 (range: 0.1 to 17.7) years. The majority of the secondary malignancies were of non-hematological origin (85.9%), most frequently skin cancer (31.0%), followed by prostate cancer (19.0%) and colorectal cancer (16.7%). Six patients (14.4%) developed hematological secondary malignancies with a predominance of B-lymphoid neoplasms. Conclusion : Long-term overall survival in HCL patients treated with sc Cld was excellent and comparable to studies using iv Cld. Despite the long follow-up, sc Cld had a curative potential and relapses requiring re-treatment were observed only in a minority of patients. Secondary malignancies were predominantly non-hematological. These data indicate that patients need to be followed carefully with a special focus on secondary malignancies. Disclosures Chalandon: Roche: Membership on an entity's Board of Directors or advisory committees, Other: Travel costs.

Objective To examine the psychometric properties of the Children's Yale-Brown Obsessive Compulsive Scales (CYBOCS) modified for pervasive developmental disorders (PDDs). Method Raters from five Research Units on Pediatric Psychopharmacology (RUPP) Autism Network were trained to reliability. The modified scale (CYBOCS-PDD), which contains only the five Compulsion severity items (range 0-20), was administered to 172 medication-free children (mean 8.2 ± 2.6 years) with PDD (autistic disorder, n = 152; Asperger's disorder, n = 6; PDD not otherwise specified, n = 14) participating in RUPP clinical trials. Reliability was assessed by intraclass correlation coefficient (ICC) and internal consistency by Cronbach's α coefficient. Correlations with ratings of repetitive behavior and disruptive behavior were examined for validity. Results Eleven raters showed excellent reliability (ICC = 0.97). The mean CYBOCS score was 14.4 (± 3.86) with excellent internal consistency (α = .85). Correlations with other measures of repetitive behavior ranged from r = 0.11 to r = 0.28 and were similar to correlations with measures of irritability ( r = 0.24) and hyperactivity ( r = 0.25). Children with higher scores on the CYBOCS-PDD had higher levels of maladaptive behaviors and lower adaptive functioning. Conclusions The five-item CYBOCS-PDD is reliable, distinct from other measures of repetitive behavior, and sensitive to change.