Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Idiopathic portal hypertension (IPH) has been reported rarely in POEMS syndrome. The precise etiopathogenesis of IPH is not fully elucidated. We report a 46-year-old male patient presenting with POEMS syndrome. He presented postprandial vomiting and left tinnitus. Loss of appetite, leanness, and excessive sweat were concomitant symptoms. Abdominal examination revealed splenomegaly and an absence of hepatomegaly. Abdominal ultrasonography was therefore performed, revealing hepatosplenomegaly with dilatation of the splenic vein. An abdominal computed tomography confirmed the presence of an 18.5 cm splenomegaly with dilatation of the splenic and portal vein. Upper endoscopy with biopsy showed minimal, non-atrophic, mildly active chronic, follicular antrofundic gastritis without esophageal varices. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with IPH. The patient initially received six courses of the CTD (cyclophosphamide, thalidomide, and dexamethasone) protocol. He subsequently proceeded to an autologous stem cell transplant (ASCT), and the patient achieved a considerable improvement. POEMS syndrome could be complicated with IPH. There are only a few cases of IPH associated with POEMS syndrome in the literature. This case highlights the manifestation of portal hypertension in POEMS syndrome.

An Unusual Presentation of Extramedullary Relapse Following Blinatumomab in Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.

In adult patients, extramedullary relapse (EMR) in B-acute lymphoblastic leukemia (B-ALL) has a pejorative prognosis, especially after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Blinatumomab, a bispecific CD3/CD19 antibody, is approved for relapsed/refractory acute lymphoblastic leukemia (ALL) and has proven its efficacy with good complete response (CR) rates and molecular responses in several trials. Unusual sites of relapse following treatment with blinatumomab for ALL are rarely reported. We describe the case of a 23-year-old male with B-ALL characterized as Philadelphia chromosome-positive without extramedullary lesions at diagnosis. He benefited from a matched-related donor allo-HSCT at first remission. A relapse in the bone marrow and central nervous system was diagnosed four months later. A treatment with blinatumomab was initiated with the obtention of CR after one cycle. During the third cycle of blinatumomab, multiple sites of EMR occurred initially with a painless swelling appearing in the areolas and the nipples, followed by bilateral testicular hypertrophy and moderate paraplegia. A diagnosis of leukemic infiltration on the areola-nipple complex was made by cytological analysis of the fine-needle aspiration of the left areola. The analysis of bone marrow was normal, but molecular BCR-ABL was positive. Systemic chemotherapy with hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and cycles of blinatumomab with nilotinib was initiated in association with intrathecal chemotherapy and whole-brain radiation therapy. Clinical, molecular, and central nervous remissions were obtained. We report this case to describe multiple sites of EMR of B-ALL with atypical breast infiltration in an adult male patient following treatment with blinatumomab.

Unusual Cutaneous Location of Langheransian Histiocytosis: A Case Report.

Langheransian cell histiocytosis (LCH) is a rare pathology characterized by the proliferation of CD1+ and Langerin+ cells. It can affect all ages, with an estimated prevalence of one to two cases/100,000 habitants. The involvement is often multi-visceral; however, isolated cutaneous involvement can be found in 40% of cases with very variable manifestations. We report the case of 45-year-old women followed for non-insulin-dependent diabetes and primary hyperparathyroidism suffering from isolated and refractory cutaneous histiocytosis.

Burkitt's lymphoma of medial part of clavicle: Case report of unusual localization.

INTRODUCTION: Burkitt lymphoma is a form of non-Hodgkin's lymphoma in which cancer starts in immune cells called B-cells. Recognized as a fast-growing human tumor, it is considered as a medical emergency requiring immediate diagnostic and therapeutic intervention. However, intensive chemotherapy can achieve long-term survival in more than half the people with Burkitt lymphoma. CASE REPORT: We report a case of Burkitt's lymphoma of medial part of clavicle was diagnosed in a 60 years year-old man and review the literature concerning the clinical features, radiological appearance, histopathological findings and treatment options. DISCUSSION: Clinical course of Burkitt lymphoma is aggressive and rapid, commonly occurs in children and young adults with frequent involvement of Bone Marrow, associated with impaired immunity and is rapidly fatal if left untreated, early diagnosis can be life saving for Burkitt lymphoma. As per the literature available this is the second case of Burkitt's lymphoma presenting primarily in medial part of clavicle.

Primary Plasma Cell Leukemia Revealed by a Mandibular Lesion: A Case Report.

Primary plasma cell leukemia (PCL) is a rare and aggressive hematological malignancy exhibiting a circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count >2000/mm3. We report a case of a 37-year-old woman presented to the Department of Hematology with a two-month history of growth inside the oral cavity in the upper jaw and weakness. The physical examination revealed a voluminous mass involving the left side of the maxillary gingiva. The maxillofacial computerized tomography (CT) scan confirmed the presence of a solid tissue mass at the left upper maxilla. A biopsy sample obtained from the lesion showed a plasma cell infiltration. The laboratory findings revealed anemia, renal impairment with high levels of creatinine and calcium. Serum protein electrophoresis found a monoclonal peak at IgG lambda, a high level of lambda free light. The diagnosis was subsequently confirmed by a peripheral-blood smear revealed 25% of plasma cells and bone marrow aspiration with 50% of plasma cell infiltration. Primary plasma-cell leukemia (pPCL) was confirmed. The patient received VTD chemotherapy (bortezomib, thalidomide, and dexamethasone) followed by autologous stem cell transplant (ASCT), which resulted in complete remission. At the six-month follow-up, the patient relapsed with extramedullary multiple lesions under ineffective rescue therapy. Response to frontline treatments may be significant initially but short-lived with a dismal median overall survival below one year. This case report aims to highlight the need for awareness among clinicians of the relevance of examining other associated clinical features of pPCL, given its aggressive course and rapid progress without the therapy.

C-reactive protein as an early predictor of COVID-19 severity.

BACKGROUND: Data for predicting severity of patients with COVID-19 infection are sparse and still under investigation. We retrospectively studied whether the admission serum C-reactive protein level (CRP) can serve as nearly predictor of disease severity during COVID-19 infection in comparison with other hematologic and inflammatory markers. METHODS: We included all consecutive patients who were admitted in Cheikh Khalifa International University Hospital, Casablanca, Morocco, between February to April 2020, with a confirmed diagnosis of COVID-19 infection using SARS-CoV-2 viral nucleic acid via RT-PCR. The complete blood count and serum CRP level were routinely measured on admission. All clinical and laboratory data of patients were collected and analyzed. The classification of the disease severity was in accordance with the clinical classification of the WHO interim guidance, and the management of patients were adapted to the national management guideline. We estimated receiver operating characteristic (ROC) curves of blood routine parameters as well as their association with COVID-19 disease severity. RESULTS: 145 COVID-19 patients were included in the study. The median age (range) was 50 (32-63) years, and 75 (51.7%) were men. 101 patients were classified in the non-severe group and 44 patients in the severe group. Based on disease severity, significant differences were observed in the age, gender, comorbidities, and respiratory symptom. Similarly, the biological analysis found significant differences for the neutrophil count, lymphocyte count, eosinophil count, and CRP level. However, according to ROC curves of these laboratory biomarkers, the AUC of CRP at 0.872 was significantly higher than all other parameters. Further, CRP was independently associated with severity of COVID-19 disease (OR = 1.11, 95% IC (1.01-1.22) and or = 1.13, 95% IC (1.04-1.23)). CONCLUSIONS: This study found that the CRP level at admission represent a simple and independent factor that can be useful for early detection of severity during COVID-19 and the easy guidance of primary care.

Extramedullary plasmocytoma relapsing at differents sites: an unusual presentation.

Extramedullary plasmacytoma (EMP) is an uncommon plasma cell neoplasm results from plasma cell proliferation and consists of monoclonal plasmacytic infiltration, without bone marrow involvement and any other systemic characteristics of multiple myeloma. EMP accounts for 3% of all plasma cell neoplasms and approximately 80% to 90% of EMP involve submucosa of the upper aerodigestive, while scrotal, dermis and retroperitoneal infiltration are very rare. There are no consensus guidelines for treatment, but EMP is highly radiosensitive, surgery may be considered for some sites, but 11 at 30% can progress in multiple myeloma. We report here an exceptional case of recurrent EMP in much localization. It's about a man 72 years old with initially testicular plasmocytoma who generalized the plasmacytic infiltration after 16 months in skin and progressively in mediastinal and retroperitoneal plasmacytoma, without any medullar and bone involvement.