EJHaem最新文献

Clinical bleeding events are reported here from 773 patients with B-cell malignancies receiving pirtobrutinib monotherapy from the phase 1/2 BRUIN study (ClinicalTrials.gov identifier: NCT03740529), either in the presence or absence of antithrombotic therapy (antithrombotic exposed [AT-E], n = 216; antithrombotic nonexposed [AT-NE], n = 557). Among the AT-E cohort, 51.9% received platelet aggregation inhibitors, 36.6% received direct factor Xa inhibitors, 18.5% received heparins, 5.6% received salicylic acid for indications other than platelet aggregation inhibition, and 2.3% received thrombolytics. Warfarin was not permitted. Any-grade bleeding/bruising events occurred in 97 patients (44.9%; 95% confidence interval [CI], 38.3–51.5) in the AT-E cohort and 181 patients (32.5%; 95% CI, 28.6–36.4) in the AT-NE cohort. Most bleeding/bruising events in both cohorts began within the first 6 months of treatment (AT-E: 65.4%; AT-NE: 72.5%). Contusion was the most common bleeding/bruising event in both cohorts (AT-E: 22.7%; AT-NE: 18.1%). Grade ≥3 bleeding/bruising events were reported in six patients (2.8%) in the AT-E cohort and 11 patients (2.0%) in the AT-NE cohort. Bleeding/bruising events requiring or prolonging hospitalization were reported in 2.3% and 1.6% of patients in the AT-E and AT-NE cohorts, respectively. No bleeding/bruising events led to pirtobrutinib dose reduction or permanent discontinuation in the AT-E cohort, and one patient (0.2%) in the AT-NE cohort experienced an event requiring dose reduction. These data support the safety of pirtobrutinib in patients requiring antithrombotic therapies.

A 31-year-old man presented to the emergency department with a 5-day history of fever, headache and lower back pain which started within 48 h of travelling from his residence in Delhi, India to the United Kingdom. Admission full blood count showed a moderate thrombocytopenia (platelets 76 × 10⁹/L) with otherwise preserved counts (haemoglobin 139 g/L; white cell count 3.8 × 10⁹/L).

A blood film demonstrated frequent abnormal lymphoid cells, shown above, with deep basophilic cytoplasms, large eccentrically placed nuclei, nucleoli and perinuclear hoff. Immunophenotyping was consistent with a population of plasmablasts; CD19+ CD10− CD20− HLADR+ CD38 (bright) and CD138 (heterogeneous) without surface light chain expression [1].

The clinical presentation was felt to be in keeping with dengue virus infection which was subsequently confirmed by detecting dengue virus RNA by RT-PCR in conjunction with a positive IgG and indeterminate IgM ELISA. The patient's condition improved with supportive treatment over the following 72 h with resolution of the thrombocytopenia and circulating plasmablasts.

Dengue is a mosquito-borne viral illness which should be suspected in a febrile traveller from an endemic region displaying suitable clinical features within 2 weeks of last possible exposure [2]. There is a strong association between acute dengue infection and polyclonal plasmablast response. Atypical plasmacytoid cells with severe thrombocytopenia in the returning traveller with fever should alert treating teams to the possibility of dengue virus infection, thereby potentially avoiding further invasive testing for a primary bone marrow pathology (Figure 1, all four panels: circulating plasmablasts present on periphral blood film. M-G-G, x100 objective).

R. Noble wrote the manuscript. A. Duguid and S. Clifford revised the manuscript.

The authors declare no conflicts of interest.

The authors received no specific funding for this work.

The information presented in this manuscript is deidentified, and there is minimal risk to the patient's privacy or confidentiality.

No material from other sources is included in this manuscript.

The authors have confirmed that informed patient consent was obtained.

Clinical trial registration is not needed for this submission.

Autoimmune hemolytic anemia (AIHA) is an acquired condition caused by autoantibody mediated destruction of erythrocytes. AIHA is classified as warm or cold depending on whether the autoantibodies involved react optimally at or below body temperature (37°C), respectively. Mixed AIHA, with features of both, is rare and clinically more severe. We report a case of mixed AIHA that was found to be the presentation of systemic lupus erythematosus (SLE). Treatment with rituximab and prednisone resulted in good response. Although more commonly associated with warm AIHA, SLE can present with mixed AIHA.

A 66-year-old woman was being monitored for severe osteoporosis. Laboratory studies showed a 6 g/dL M spike and immunoglobulin M kappa paraprotein. Also, CRAB criteria were met.

The bone marrow (BM) aspirate revealed the presence of 30% atypical plasma cells (PC), which contained numerous large cytoplasmic azurophilic granules that appeared as dots resembling intracellular microorganisms. Concurrently, these cells were reminiscent of the “storage-type” histiocytes reported in lysosomal storage diseases (Figure 1, Panels A and B; May-Grunwald Giemsa stain [MGG], x100 objective). However, a subset of PC displayed coarse azurophilic granules, morphologically consistent with Snapper-Schneid bodies (Figure 1, Panels A and B [arrows]; MGG stain, x100 objective). Flow cytometry of the BM aspirate revealed a monotypic CD38+/CD138+ PC population with an aberrant profile characterized by the loss of CD45 and CD19. These PC were also CD56-, CD200+, CD20+, CD117+, and CD27- (Figure 1, Panel C), and expressed kappa immunoglobulin light chain, consistent with the observed paraprotein.

Various types of cytoplasmic inclusions have been documented in plasma cell neoplasms, including Russell bodies, crystals, and Auer rod-like inclusions, while azurophilic granules and Snapper-Schneid bodies remain uncommon. This myeloma case is particularly noteworthy as it demonstrates the presence of both azurophilic granules and Snapper-Schneid bodies in an untreated patient with a rare paraprotein: immunoglobulin M kappa. Previous case reports have documented this phenomenon in pretreated patients, including those involving diamidine treatment [1].

The presence of PC with atypical granules and an aberrant immunophenotype underscores diagnostic complexity, requiring thorough morphological and immunological examination. Careful distinction of these features from those of microbial infections or lysosomal storage diseases is essential to ensure appropriate clinical management.

Radu Chiriac wrote the manuscript; Lucile Baseggio and Luc-Marie Gerland conducted the cytological and flow cytometric studies. All authors contributed to the final manuscript.

The authors declare no conflict of interest.

The authors received no specific funding for this work.

This manuscript respects the ethical policy of Hospices Civils de Lyon for the treatment of human research participants.

No patient-identifying data were used. The authors did not obtain written informed consent from the patient but the patient did not object to his data being used for research purposes (as required by the ethics policy of Hospices Civils de Lyon).

The authors have confirmed clinical trial registration is not needed for this submission.