Hematology, Transfusion and Cell Therapy最新文献

Objective

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm arising from the olfactory neuroepithelium. It accounts for 3–5% of all nasal and Sinonasal malignancies, with an incidence of approximately 0.4 cases per million. A complete surgical resection of tumor followed by a full course of radiotherapy, is considered the treatment modality of choice for most ONBs. We aim to assess the impact of aggressive salvage radiotherapy in olfactory neuroblastoma on local recurrence and overall survival.

Case report

A 41-year-old Libyan female presented in 2020 with a mass in the right nasal cavity that caused persistent nasal congestion with intermittent epistaxis over one year ago. Histopathological characteristics and immunohistochemical findings of the biopsy confirmed an olfactory neuroblastoma grade III, Radiological imaging evaluation revealed group B stage, and an incomplete excision was done, followed by radical radiotherapy with 70 GY in 35 fractions over 5 weeks to the residual disease.

Methodology: Imaging follow-up for three years up to February 2024 shows no signs of local recurrence or distant metastasis.

Results

Although multi-disciplinary care is required, surgical treatment alone is effective for low-grade tumors with free margins. Adjuvant radiation is used for low-grade tumors with close margins, residual disease, or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Because recurrence can occur after 5 or even 10 years, aggressive management and long-term follow-up are mandatory.

Conclusion

Multimodal therapy, including post-operative radiotherapy of high-grade incompletely resected ONB, with precise treatment planning based on CT simulation, could achieve an excellent local control rate with acceptable toxicity and reasonable overall survival for patients with ONB. Still, the rarity of the disease makes it difficult to draw definitive conclusions about the role of systemic treatment in induction and concomitant settings.

Objective

Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of non-Hodgkin lymphoma (NHL). Extra nodal involvement of B-cell lymphoma is usually seen in the gastrointestinal system, followed by the skin. Skin involvement of B-cell lymphomas can be primary or secondary. In this article, we aimed to present a case of DLBCL which did not have skin involvement before but showed recurrence with skin involvement.

Case report

A 77-year-old male patient presented with a diagnosis of DLBCL based on excisional LAP biopsy in the inguinal region conducted in November 2022. Laboratory tests revealed Hgb 14.3 g/dL, WBC 4.6 × 10^3/μL, plt 191 × 10^3/μL. Following 4 cycles of R-mini CHOP based on the stage 4 DLBCL diagnosis from PET-CT, interim PET-CT showed regression in existing lesions.

Methodology: The R-miniCHOP regimen was completed with 8 cycles. In December 2023, a nodular lesion with raised erythematous ground and vascularity in the temporal region was identified (Figure 1). Dermatological evaluation and biopsy revealed infiltration consistent with high-grade B-cell lymphoma. PET-CT detected increased FDG uptake (SUVmax: 10.13) in a soft tissue-density lesion in the right parietal region. Due to age and performance status, the patient was planned for Rituximab-Lenalidomide protocol.

Results: Starting from the 1st cycle, lesions showed regression, and by the 2nd week of the 1st cycle, complete disappearance of lesions was observed (Figure-2).

Conclusion: In conclusion, while NHL usually recurs in the same sites of involvement, widespread secondary cutaneous involvement has also been reported in the literature. In our patient who did not have primary skin involvement, disease recurrence occurred in the cutaneous region.In cases like ours, the optimal treatment option is salvage chemotherapy followed by autologous stem cell transplantation.

Objective

Dermatitis artifacta is a condition in which skin lesions are produced solely by the patient's own actions. This often occurs as a result or manifestation of a psychological problem (1,2). In immune thrombotic purpura (ITP), a condition characterized by a low level of platelets, petechial rashes usually occur. Patients usually seek help for these skin manifestations (3).

Case report

A 40-year-old female patient was being followed up in the hematology clinic due to ITP. White blood count was 5.59 × 10^3/µL, hemoglobin value was 10.3 g/dL, platelet count was 21 × 10^3/µL. Peripheral smear: He was hospitalized with complaints of a low platelet count and bleeding from lesions on his arms and legs. The patient had irregularly shaped lesions and bleeding areas on both forearms and legs.

Methodology: The patient was hospitalized due to hematological ITP, but these skin lesions were not compatible with ITP. A psychiatrist was consulted as the patient attempted to draw attention to her lesions during daily visits. She was diagnosed with factitial dermatitis by psychiatry.

Results

Later, upon the development of symptoms such as epistaxis and hemoptysis associated with ITP, the patient's attention was directed to the newly developing symptoms, and the effort to create lesions decreased and the existing lesions were observed to regress.

Conclusion

An autoantibody-mediated thrombocytopenic condition called immune thrombocytopenic purpura (ITP) causes an accelerated loss of platelets and presents with petechial rashes (4). On the other hand, dermatitis artifacta is a psychological problem that is characterized by self-induced skin lesions and should be examined accordingly (5). Clinicians should always be aware that skin lesions in ITP patients may be oriented toward psychological disorders.

This case study examines a 55-year-old male without previously known comorbidities, who was evaluated due to palpable lymph nodes identified incidentally in the neck, inguinal, and axillary regions. The extensive diagnostic work-up, including advanced imaging, revealed a pattern not commonly associated with diffuse large B-cell lymphoma (DLBCL), including hypermetabolic thickening in the posterior nasopharynx, significant hypermetabolism around the pancreas, and suspicious activity in the spleen and lung. Notably, the involvement extended to both parotid glands and a vast array of lymph nodes, marking an atypical presentation that underscores DLBCL's potential for widespread disease. Biopsies confirmed DLBCL with a non-germinal center phenotype, an aggressive variant with implications for treatment and prognosis. Despite a thorough diagnostic process, the patient elected to forgo the recommended DA-R-EPOCH chemotherapy, highlighting significant ethical and autonomy considerations within the realm of oncological care. This case contributes to the medical literature by illustrating the diagnostic challenges and treatment decision complexities in cases of DLBCL with unusual disease distribution and patient care preferences.

Objective

Multiple myeloma (MM) is a heterogeneous disease with the uncontrolled clonal proliferation of plasma cells, accounting for approximately 10% of all hematologic cancers . Hence without curative therapy, the treatment aims to improve overall survival.Pomalidomide (POM) is a third-generation immunomodulatory agentPomalidomide can be administered with dexamethasone or in combination with proteasome inhibitors (bortezomib) and monoclonal antibodies (isatuximab, daratumumab). We retrospectively analysed all patients treated with pomalidomide at our centre between 2017 and 2023.

Methodology

All patients who had received or were currently receiving treatment with pomalidomide at Ege University Hematology Outpatient Clinic between January 2017 and April 2023 were included. To be included in response assessments, patients had to have measurable disease as defined by International Myeloma Working Group (IMWG) guidelines (Kumar et al, 2016) and have completed at least one cycle of pomalidomide with repeat biomarkers performed. Treatment consisted of 28-day cycles of pomalidomide (taken daily on days 1–21) plus dexamethasone (on days 1, 8, 15 and 22), plus or minus a third agent.

Results

A total of 25 patients who received treatment with pomalidomide were identified. Of these, 24 were able to be included in response analyses. Of the remaining 1 patient for whom response could not be assessed,had an anaphylactoid reaction with pomalidomide and did not complete a single cycle of treatment.

The analysis includes a total of 23 patients with RRMM, 1 patient with newly diagnosed multipl myeloma who had central nervous system involvement at diagnosis. 23 patients treated with POM-DEX in the lines of therapy subsequent to the second (third to seventh) line. Median patient age at diagnosis was 55 years (range 42–82), 7 (28%) patients were 65 or older than 65 years old. 13 patients were male (54,25%) and 11 were female (45,85%). 6 (25%) patients had International Staging System (ISS) stage I, 5 (20,8%) had stage II, 11 (45,8%) stage III myeloma, respectively (2 patients had not adjusted) stage III myeloma. 79,2 % (n=19)of patients had IgG, 4,2% (n=1) had IgD, 79,2 % (n=19) had kappa and 20,8 % (n=5) had lambda subtype myeloma. Six patients (25 %) had extramedullary disease and 18 (75 %)had lytic bone lesions at diagnosis.

Moreover, 12 (%50)patients had received a previous autologous stem cell transplant (single or double). 1 patient had autologous stem cell transplant after pomalidomide therapy. On data cut off (1 August 2023), median survival from initial diagnosis was not reached .Nearly all patients had received at least two previous lines of therapyand, as per guideline, had been exposed both to lenalidomide and bortezomib. Efficacy In a total of 24 patients, the treatment response rate (ORR), including all patients with a partial response or better, was 41.7%. A total of 10 pa