Pleural effusion,ascites,colon ulcers and hematochezia:What we can learn from the diagnostic process of a patient with plasma cell myeloma:A case report

BACKGROUND Plasma cell myeloma(PCM)is characterized by hypercalcemia,renal impairment,anemia,and bone destruction.While pleural effusion,ascites,abdominal pain,and bloody stool are common manifestations of lung disease or gastrointestinal disorders,they are rarely observed in patients with PCM.CASE SUMMARY A 66-year-old woman presented with complaints of recurrent chest tightness,wheezing,and abdominal bloating accompanied by bloody stools.Computed tomography revealed pleural effusion and ascites.Pleural effusion tests showed inflammation,but the T-cell spot test and carcinoembryonic antigen were negative.Endoscopy showed colonic mucosal edema with ulcer formation and local intestinal lumen stenosis.Echocardiography revealed enlarged atria and reduced left ventricular systolic function.The diagnosis remained unclear.Further testing revealed elevated blood light chain lambda and urine immunoglobulin levels.Blood immunofixation electrophoresis was positive for immunoglobulin G lambda type.Smear cytology of the bone marrow showed a high proportion of plasma cells,accounting for about 4.5%.Histopathological examination of the bone marrow suggested PCM.Flow cytometry showed abnormal plasma cells with strong expression of CD38,CD138,cLambda,CD28,CD200,and CD117.Fluorescence in situ hybridization gene testing of the bone marrow suggested 1q21 gene amplification,but cytogenetic testing showed no clonal abnormalities.Colonic mucosa and bone marrow biopsy tissues were negative for Highman Congo red staining.The patient was finally diagnosed with PCM.CONCLUSION A diagnosis of PCM should be considered in older patients with pleural effusion,ascites,and multi-organ injury.

Persicaria lanigera (Polygonaceae) leaf extract exhibits antiulcerogenic and antiproliferative activities against acetic acid-induced ulcerative colitis and cotton pellet-induced granuloma tissue in rats

Objective:To assess the effect of leaf extract of Persicaria lanigera on cotton pellet-induced granuloma tissue formation and acetic acid-induced ulcerative colitis.Methods:Rats were randomly divided into six groups:normal control,negative control,positive control(dexamethasone or sulfasalazine)as well as Persicaria lanigera(100-600 mg/kg)-treated groups.The effects of the extracts on body weight,antioxidant,and hematological parameters,as well as mast cell proliferation,were assessed.In addition,a histological evaluation was conducted.Results:Persicaria lanigera extract significantly decreased the mean exudate amount and suppressed granuloma tissue formation in a concentration-dependent manner in rats(P<0.05).Additionally,the extract significantly increased body weight,improved hematological profile,reduced the disease activity index score and malondialdehyde level,as well as enhanced catalase and superoxide dismutase activities(P<0.05).Histological evaluation showed Persicaria lanigera extract alleviated acetic acid-induced colonic damages,as evidenced by decreased cell necrosis,edema,and inflammatory cell infiltration.Conclusions:Persicaria lanigera extract possesses antiproliferative,antioxidative,and anti-colitis activities.However,its underlying mechanisms of action need further investigation.

Bortezomib-Induced Bilateral Eye Swelling and Cutaneous Adverse Reaction in a Patient with Plasma Cell Leukemia—A Case Report

Bortezomib, a proteasome inhibitor, is an established therapy against plasma cell leukemia—a variant of plasma cell dyscrasias. Its most frequent side effects have been listed as peripheral neuropathy, neuropathic pain, thrombocytopenia, and gastrointestinal problems. Allergic skin reaction is a rarely documented side effect in patients receiving bortezomib-based chemotherapy. A combination therapy consisting of intravenous bortezomib, oral Revlimid tablets, and oral dexamethasone tablets has been prescribed for the patient after his recent diagnosis of plasma cell leukemia. While receiving his third treatment cycle, he developed an allergic reaction (skin rash) involving the neck, and wrists, and mild bilateral eye swelling. The infusion was stopped immediately and then ciprofloxacin ophthalmic solution and oral diphenhydramine 25 mg were prescribed to the patient with significant improvement in his clinical condition. He was temporarily taken off bortezomib. At a follow-up visit a week later, a significant improvement was noticed in his condition. Rash had reduced on neck and wrists, and eye swelling had reduced as well. As of the time of writing this case report, he has been temporarily taken off bortezomib, but other medications in the treatment regimen were continued as prescribed.

From Myeloma to Plasma Cell Leukemia, Persistent Inequalities

Multiple myeloma (MM) is both a complex and heterogeneous disease. Cytogenetic and molecular abnormalities lead to resistance to treatment and transformation to plasma cell leukemia, which is defined by the presence in circulating blood of plasma cells over 2 G/L, or more than 20% of leukocytes. It is an uncommon hematological malignancy with a poor prognosis. Against this backdrop, we report an observation of multiple myeloma transformed into plasma cell leukemia diagnosed at the Hôpital Principal de Dakar (HPD) that occurred on a 64-year-old man with a history of thyroidectomy followed for multiple myeloma presenting with Salmon et Durie stage IIIA and ISS stage I. Despite a marked improvement in management strategy, myeloma remains an almost invariably incurable disease. However, the development of genetic and molecular biomarkers is necessary to improve its prognosis.

Multiple Myeloma: Modification and Adaptation of Plasma Cell Morphology Algorithm for the Prognosis of Congolese Patient

Objectives: To determine the multiple myeloma (MM) prognostic formulas applicable in Congolese with many aberrant and/or immature plasma cells according to GOASGUEN’s plasma cell morphology algorithm (PMA). Methods: The prospective, observational and descriptive study was carried out at the Ngaliema Clinic in Kinshasa from July 2016 to December 2019. The variables studied were the plasma cell types and the prognosis groups according to GOASGUEN and ZANDECKI. The nucleolus, the chromatin and the N/C ratio of 2 for each plasma cell encountered in multiple myeloma are examined in order by asking three successive questions as follows: 1) Is the nucleolus present? If yes, we denote 1;if not, note 0;2) Is the chromatin thin? If yes, we score 1;if not we score 0;3) The N/C ratio is greater than 6, if so, 1 is noted;if not, we note 0. We obtain a number with 3 successive digits preceded by the letter P for each plasma cell. Results: The overall rate of immature plasma cells and aberrant plasma cells in MM among Congolese was high at 26.6%. Their integration in the prognostic formulas showed for group A" = 22 patients, group B" = 5 patients and group C" = 32 patients. Examination of plasma cell types in the deceased showed that 14/17 had a P001 plasma cell count ≥ 15%. Conclusion: The high rate of aberrant and/or immature plasma cells, the P001 plasma cell type at a rate ≥ 15%, found mostly in group C" with poor prognosis, are responsible for the aggressive nature of MM in Congolese Blacks.

Perifollicular granulomas with Ig G4 plasmacytosis:A case report and review of literature

Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4(IgG 4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells(at least 80% of Ig G+ plasma cells were positive for IgG 4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG 4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E(> 23000 IU/mL) and slight increase of total Ig G, but normal serum Ig G4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, Ig G4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.

Autoimmune pancreatitis with IgG4-positive plasma cell infiltration in salivary glands and biliary tract

A 62-year-old male was referred to our hospital because of liver dysfunction, diffuse pancreatic swelling, and trachelophyma. At admission, the patient was free of pain. Physical examination showed enlarged and palpable bilateral submandibular masses, but no palpable mass or organomegaly in the abdomen. Laboratory findings were as follows： total protein 90 g/L with γ-globulin of 37.3% （33 g/L）, total bilirubin 4 mg/L, aspartate aminotransferase 39 IU/L, alanine aminotransferase 67 IU/L,γ-glutamyl transpeptidase 1 647 IU/L, and amylase 135 IU/L. Autoantibodies were negative, and tumor markers were within the normal range. Serum IgG4 level was markedly elevated （18 900 rag/L）. Computed tomography （CT） showed diffuse swelling of the pancreas and dilatation of both common and intra-hepatic bile ducts. Endoscopic retrograde pancreatography （ERP） revealed diffuse irregular and narrow main pancreatic duct and stenosis of the lower common bile duct. Biopsy specimens from the pancreas, salivary gland and liver showed marked periductal IgG4-positive plasma cell infiltration with fibrosis. We considered this patient to be autoimmune pancreatitis （AIP） with fibrosclerosis of the salivary gland and biliary tract, prescribed prednisolone at an initial dose of 40 mg/d. Three months later, the laboratory data improved almost to normal. Abdominal CT reflected prominent improvement in the pancreatic lesion. Swelling of the salivary gland also improved. At present, the patient is on 10 mg/d of prednisolone without recurrence of the pancreatitis. We present here a case of AIP with fibrosclerosis of salivary gland and biliary tract.

Contrast-enhanced multidetector computed tomography features and histogram analysis can differentiate ameloblastomas from central giant cell granulomas

BACKGROUND No qualitative or quantitative analysis of contrast-enhanced computed tomography(CT)images has been reported for the differentiation between ameloblastomas and central giant cell granulomas(CGCGs).AIM To describe differentiating multidetector CT(MDCT)features in CGCGs and ameloblastomas and to compare differences in enhancement of these lesions qualitatively and using histogram analysis.METHODS MDCT of CGCGs and ameloblastomas was retrospectively reviewed to evaluate qualitative imaging descriptors.Histogram analysis was used to compare the extent of enhancement of the soft tissue.Fisher’s exact tests and Mann–Whitney U test were used for statistical analysis(P<0.05).RESULTS Twelve CGCGs and 33 ameloblastomas were reviewed.Ameloblastomas had a predilection for the posterior mandible with none of the CGCGs involving the angle.CGCGs were multilocular(58.3%),with a mixed lytic sclerotic appearance(75%).Soft tissue component was present in 91%of CGCGs,which showed hyperenhancement(compared to surrounding muscles)in 50%of cases,while the remaining showed isoenhancement.Matrix mineralization was present in 83.3%of cases.Ameloblastomas presented as a unilocular(66.7%),lytic(60.6%)masses with solid components present in 81.8%of cases.However,the solid component showed isoenhancement in 63%.No matrix mineralization was present in 69.7%of cases.Quantitatively,the enhancement of soft tissue in CGCG was significantly higher than in ameloblastoma on histogram analysis(P<0.05),with a minimum enhancement of>49.05 HU in the tumour providing 100%sensitivity and 85%specificity in identifying a CGCG.CONCLUSION A multilocular,lytic sclerotic lesion with significant hyperenhancement in soft tissue,which spares the angle of the mandible and has matrix mineralization,should indicate prospective diagnosis of CGCG.

Venetoclax in combination with chidamide and dexamethasone in relapsed/refractory primary plasma cell leukemia without t(11;14):A case report

BACKGROUND Conventional therapies for primary plasma cell leukemia(pPCL)are usually ineffective,with a short remission time with the use of multiple myeloma medications,showing aggressiveness of pPCL.B-cell lymphoma-2 inhibitor venetoclax is usually used for relapsed/refractory multiple myeloma(RRMM)with t(11;14).There are very few studies published on the use of venetoclax in pPCL without t(11;14).Similarly,histone deacetylase inhibitors are considered effective for the treatment of RRMM,but there are no reports on their use in pPCL.CASE SUMMARY A 57-year-old woman with severe anemia,thrombocytopenia,multiple bone destruction,impaired renal function,and 42.7%of peripheral plasma cells is reported.After multiple chemotherapy regimens and chimeric antigen receptor Tcell treatment,the disease progressed again.The patient had very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.CONCLUSION The success of venetoclax-chidamide-dexamethasone combination therapy in achieving a very good partial response suggested that it can be used for refractory/relapsed pPCL patients who have been exhausted with the use of various drug combinations and had poor survival outcomes.

A Case Report of a Peripheral Giant Cell Granuloma in a Patient on Forteo

Peripheral giant cell granuloma (PGCG) is a common reactive gingival lesion that histologically resembles the central giant cell granuloma, and is a central lesion of the jaws. Occasionally, central giant cell granuloma-like lesions may be seen in association with hyperparathyroidism. Rarely, PGCG-like lesions have been described in a background of hyperparathyroidism. We describe the case of a 91-year-old woman taking teriparatide for the treatment of osteoporosis and presented with a peripheral giant cell granuloma of the mandibular posterior area.

Surgical Treatment of Central Giant Cell Granuloma with Carnoy’s Solution

A 25-year-old woman presented a unilateral mandibular swelling, after laboratory analysis, radiological studies and aspiration puncture biopsy, the pathological diagnosis of Central Giant Cell Granuloma was made. Radiographically, the multilocular lesion involved the left lower first molar and was 30 mm long by 20 mm high. Dental extraction of the molar involved with forceps and exeresis in toto of the tumor were performed using a Volkmann cutting curette. The tumor made up of 90% vascular tissue produced intra-surgical bleeding that made visibility difficult. The bone bed curettage was aggressive except for its base in direct contact with the inferior dental nerve. Subsequently, to avoid recurrence, the surgical protocol includes a double irrigation with Carnoy’s solution, (fixative composed of 60% ethanol, 30% chloroform and 10% glacial acetic acid 98%) for 5 minutes that produces chemical cauterization. Finally, it was washed with physiological solution and the cavity was filled with iodoformed gauze. The gauze was exchanged and removed gradually over three weeks for second intention healing. This case is presented to demonstrate that inexpensive chemical treatment can be safely used for outpatient surgery under local anesthesia. Because Carnoy’s solution achieved control of intraoperative bleeding, allowed bone healing and prevented recurrence of the lesion in clinical radiographic follow-up for 15 years.

Haploidentical stem cell transplantation used in treating primary plasma cell leukemia: a case report

Here we report a successful protocol in treatment of a patient with primary plasma cell leukemia (PPCL) using haploidentical stem cell transplantation (hi-HSCT). During first complete remission after routine chemotherapy, the patient received autologous blood stem cell transplantation, but he had relapse later. He gained a second CR after chemotherapy and underwent hi-HSCT from his daughter, who had HLA mismatched at three loci. Recovery of hemopoiesis was found at day 14 and complete donor chimerism was confirmed by PCR-STR on day 34, 95 and 238. The patients have survived disease-free for 56 months since hi-HSCT, without serious graft-versus-host-disease.

Unusual aggressive behavior of central giant cell granuloma following tooth extraction

Central giant cell granuloma(CGCG) is found exclusivelyin jaws. Its etiopathogenesis is unclear; however it is suggested that it can arise as a reactive response to trauma. This report describes an aggressive variety of CGCG which raises a question; can extraction of tooth modify the behavior of CGCG? A 46 years old male had reported with a rapidly increasing intraoral and extraoral swelling of lower jaw following tooth extraction. Radiographic examination revealed a large multilocular lesion involving the body and ramus of mandible which had been proved to be aggressive CGCG on histopathological examination. The importance of radiographic examination prior to extraction of teeth and importance of inclusion of CGCG in jaw swellings associated with mobility of teeth or failure of healing sockets is emphasized.

PRIMARY PLASMA CELL LEUKEMIA(A COMPREHENSIVE ANALYSIS OF 44 CASES)

Four cases of primary plasma cell leukemia (PPCL) admitted to Zhongshan Hospital from 1959 to 1987 are reported with a review on additional 40 cases reported in China. Comparing with the 57 cases of multiple myeloma (MM) treated in our hospital, the following features were observed in PPCL: (1) The age was younger, with a mean of 45.2 years, 34.1% of the patients were under 40 years. (2) Onset was abrupt. Duration from onset to diagnosis was 2 months or less in 77% patients but never beyond 6 months. (3) 81.8% patients had liver enlargement, 59.1% splenomegaly and 61.4% sternum tenderness. (4) All patients showed marked anemia with an average hemoglobin of 65 g/L. BPC count was less than 100 × 109/L in 76% patients and WBC was more than 10×109/L in 77%. (5) Plasma cell number in the marrow was markedly increased with an average of 69%, of which the blast cells and immature forms were predominant. (6) No destruction of bones was shown on X-ray film in 68.3% patients. (7) The response to chemotherapy was poor with a total response rate of 18% and a mean survival of 2 months. All the above-mentioned clinical features were significantly different from those of MM. In addition, these two diseases were also different in cytology, cytogenetics and ultrastructure. Therefore, PPCL should be considered as a special type of acute leukemia distinct from MM. High dose of alkylating agents in combination with autologous bone marrow transplantation might improve the prognoses.

Refractory gastric ulcer with abundant IgG4-positive plasma cell infiltration: A case report

We describe a 77-year-old man with refractory gastric ulcer that worsened after Helicobacter pylori eradication therapy.Pathology showed marked infiltration of IgG4-positive plasma cells in the gastric lesions,which led us to suspect IgG4-related sclerosing disease.To the best of our knowledge,this is the first report of IgG4-related gastric ulcer without the main manifestation of autoimmune pancreatitis.

Recurrent hepatitis C virus after transplant and the importance of plasma cells on biopsy

Hepatitis C virus(HCV) is the leading indication for liver transplantation in the United States.It recurs universally after transplant but the rate of fibrosis and the development of graft failure is variable.Different donor and recipient features have been demonstrated to impact fibrosis.Plasma cell hepatitis,a histologic finding,is one feature associated with poor graft and patient outcomes.The pathogenic mechanism resulting in plasma cell hepatitis is poorly understood,with evidence suggesting a role for both the HCV and the immune system.A recent publication described plasma cell hepatitis in a larger context of immune medicated graft dysfunction in transplant recipients receiving interferon based therapy.This manuscript will highlight the topic of plasma cell hepatitis and provide commentary on the lack of recognition,the data regarding pathophysiologic mechanisms and the potential management options.

Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells

Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin(30.1 g/L, range: 35.0-55.0 g/L), serum potassium(2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose(2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h(range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG 4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.

The new proof of neuro-endocrine-immune network-expression of islet amyloid polypeptide in plasma cells in gastric mucosa of peptic ulcer patients

INTRODUCTION Peptic ulcer,as a common disease,seriouslyaffected people’s,work and life.Its occurrence,development and change have close relationshipwith the change of people’s moods.Animalexperiment proved that significant changes occurredin the endocrine system of the gastric ulcer rats.

Gastrointestinal bleeding as initial presentation of extramedullary plasma cell neoplasms: A case report and review of the literature

BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.

Construction of Larger Area Density-Uniform Plasma with Collisional Inductively Coupled Plasma Cells

The plasma density and electron temperature of a multi-source plasma system composed of several collisional inductively coupled plasma （ICP） cells were measured by a doubleprobe. The discharges of the ICP cells were shown to be independent of each other. Furthermore, the total plasma density at simultaneous multi-cell discharge was observed to be approximately equal to the summation of the plasma density when the cells discharge separately. Based on the linear summation phenomenon, it was shown that a larger area plasma with a uniform density and temperature profile could be constructed with multi-collisional ICP cells.