Pigmented villonodular synovitis and rheumatoid arthritis: diagnostic challenges and therapeutic considerations in a case of knee pain.

We present a case of a woman in her 30s who visited the rheumatology clinic due to her persistent knee pain for 5 years, which spread to multiple joints. She was diagnosed with seropositive rheumatoid arthritis (RA). While most joints responded well to methotrexate and subsequently etanercept, persistent unilateral knee pain prompted further investigation. Imaging revealed synovitis and joint effusion in her knee, prompting arthroscopy and synovial biopsy, revealing pigmented villonodular synovitis (PVNS). Despite initial success with a tricompartmental synovectomy, her disease recurred. The decision was made to pursue medical therapy, with pexidartinib initiated by the oncology team. Our case report highlights the importance of considering other underlying conditions in patients with RA who do not achieve full clinical improvement despite standard treatment. Physicians should remain vigilant for atypical presentations and imaging features in patients with RA, for early recognition of PVNS can significantly impact treatment decisions and patient outcomes.

Massive arterial and venous thrombosis from smouldering multiple myeloma: further evidence for monoclonal gammopathy of thrombotic significance.

A man in his 40s presented to the emergency department after 2 weeks of abdominal pain and bloating. Radiological investigations revealed multiple unusual sites of thrombosis, including large thrombi in his portal and mesenteric veins, and a left ventricular thrombus with resultant embolic infarcts to his spleen, kidneys, coronary arteries and brain. Standard causes of underlying thrombophilia were excluded. A serum protein electrophoresis and serum-free light chains, with subsequent bone marrow biopsy, lead to the diagnosis of smouldering multiple myeloma (sMM), albeit an unusual presentation with severe clinical sequelae. Although sMM is known to be associated with an increased risk of venous thromboembolism, it is not recognised to cause thrombosis in both venous and arterial vascular beds simultaneously. Physicians encountering patients with multiple thrombi in unusual vascular beds without clear aetiology should consider an underlying monoclonal gammopathy in their list of differentials.

Cardiovascular collapse and McConnell's sign as early manifestations of leucostasis.

A man in his late 70s with chronic myelomonocytic leukaemia presented for evaluation of acute leukaemic transformation and initiation of cytoreductive therapy after being found to have asymptomatic hyperleucocytosis. Within 24 hours, the patient developed vasopressor-refractory shock, severe lactic acidosis and multiorgan failure. Serial echocardiographic assessments revealed interval enlargement of the right ventricle with development of the McConnell's sign, and abdominal CT showed diffuse bowel wall thickening, likely due to ischaemia. CT angiography excluded pulmonary embolism or occlusion of intra-abdominal arteries. Despite aggressive care, the patient died from cardiovascular collapse within 8 hours of the onset of hypotension. An autopsy revealed extensive infiltration of early myeloid cells in pulmonary, myocardial, hepatic and intestinal microvasculature. This case illustrates different mechanisms by which leucostasis causes acute cardiovascular collapse and stresses the emergent nature of this diagnosis.

Acute kidney failure reveals primary renal non-Hodgkin lymphoma.

A male patient in his 60s was admitted to our hospital with symptoms of dyspnoea, asthenia, diaphoresis and acute kidney failure. No tumour or infection was detected in initial screening. However, laboratory examination suggested that the acute kidney failure was due to an intrarenal cause, exhibiting a tubular injury pattern and indications of tumour lysis syndrome. Initial hydration therapy, paired with intravenous rasburicase, rapidly improved the kidney function. Unfortunately, the kidney function deteriorated once again, prompting a kidney biopsy that revealed an aggressive diffuse large B-cell non-Hodgkin lymphoma of the kidney. The chemotherapy, comprised of R-CHOP scheme, led to a full recovery of the kidney function and complete remission of the lymphoma. Primary renal non-Hodgkin lymphoma without nodal manifestation is rare, and its pathophysiology is poorly understood. Therapy schemes can vary significantly between cases, relying primarily on non-renal-specific haemato-oncological guidelines. Therefore, further studies are needed to develop the best therapeutic approaches.

Extranodal diffuse large B-cell lymphoma presenting with extensive organ involvement.

Extranodal involvement in diffuse large B-cell lymphoma (DLBCL) is defined as disease outside of the lymph nodes and occurs in up to one-third of patients, though multiorgan extranodal involvement is rare. Here, we describe a case of a patient presenting with widely metastatic lesions, including involvement of the lung, parotid gland, breast, pancreas, femur and multiple soft tissue masses, with initial concern for primary breast malignancy. Breast pathology and imaging were consistent with triple-expressor, double-hit stage IV high-grade B-cell lymphoma with extensive extranodal involvement. Extranodal involvement is a poor prognostic factor associated with high rates of treatment failure, and novel therapies targeting CD19 are currently being studied for relapsed and refractory DLBCL. Extranodal disease is a complex entity that can involve virtually any organ system and should be considered for new presentations of malignancy.

IgA plasma cell myeloma presenting as cold agglutinin-induced haemolytic transfusion reaction.

Cold agglutinins produced in the setting of B cell neoplasms, such as lymphoplasmacytic lymphoma and plasma cell myeloma, can mediate autoimmune haemolytic anemia. Transfusion of these patients can exacerbate cold agglutinin-mediated haemolysis. Moreover, the workup for these reactions represents a diagnostic challenge due in part to false negative direct antiglobulin tests (DATs). Here, we report an anaemic patient who after a red blood cell (RBC) transfusion performed without blood warming, experienced a DAT-negative haemolytic transfusion reaction, and was later diagnosed with IgA-multiple myeloma, which showed an uncommon granular pattern by CD138 immunohistochemistry. Extensive workup excluded other diagnostic possibilities, including the presence of Donath-Landsteiner antibodies and cryoglobulins. Successful treatment with CyBorD (cyclophosphamide, bortezomib and dexamethasone) achieved complete remission, and additional RBC transfusions using warmers were completed uneventfully.

Subcutaneous panniculitis-like T-cell lymphoma: fever and facial swelling with hemophagocytic syndrome.

We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and hemophagocytic syndrome (HPS) on evaluation. The histopathological examination of skin punch biopsy from the face and chest wall showed SPTCL. Given the associated HPS, he was started on steroid and multidrug chemotherapy following which he had symptomatic improvement.

An interesting case of AL amyloidosis and MM: a complex scenario with cardiac involvement.

Our patient presented with complaints of progressive shortness of breath for 1 month. She was diagnosed with a case of infiltrative type of restrictive cardiomyopathy (RCM) based on echocardiography and cardiac MRI findings. Her fat pad biopsy was suggestive of AL type of amyloidosis (AL). She was diagnosed with a case of multiple myeloma (MM) based on bone marrow biopsy findings with 48% plasma cells and a skeletal survey with lytic bone lesions on the skull, thus meeting the Crab criteria. We want to highlight the complex nature of this case and the difficulties associated with making a diagnosis. This case report presents an excellent opportunity to touch on the interesting topics of RCM, amyloidosis and MM.

Extramedullary plasmacytoma with associated multiple myeloma as a presentation of the posterior mediastinal mass: a rare clinical encounter.

A plasmacytoma is a single, isolated tumour of abnormal plasma cells. It can develop within the bone, known as solitary plasmacytoma of bone, or outside the bone, referred to as extraosseous (extramedullary) plasmacytoma, without spreading to other parts of the body. Plasmacytoma, an uncommon presentation in the posterior mediastinum, usually arises as solitary or multiple lesions in bone or soft tissues. The standard treatment involves definitive radiotherapy, potentially curative for extramedullary cases. The prognosis varies, being more favourable without concurrent multiple myeloma and worsening with high-risk cytogenetics. The case involves a male in his early 80s with an extensive medical history presenting with difficulty swallowing and dyspnoea. The diagnosis revealed a rare posterior mediastinal plasmacytoma associated with multiple myeloma, emphasising the importance of prompt diagnosis and treatment.

Possible case of bortezomib-induced ileus paralytic.

This report describes a case of a patient with active multiple myeloma who was started on bortezomib, cyclophosphamide and dexamethasone and subsequently presented to the emergency department with acute intestinal obstruction one week later. The patient underwent exploratory laparotomy, but no mechanical cause of the obstruction was found. The patient later developed sepsis and eventually died. The possible cause of the intestinal obstruction was attributed to bortezomib, and the paper discusses the potential mechanism of this side effect and its management based on available literature.

Passenger lymphocyte syndrome following minor ABO-mismatched liver transplantation.

Passenger lymphocyte syndrome is an immunologic disorder observed in solid organ and haematopoietic stem cell transplantation in which B lymphocytes within a donor graft are transferred to the recipient and subsequently produce circulating antibodies against host red blood cell antigens. The syndrome is most likely to occur in minor ABO blood group mismatched or Rh incompatible transplantation. Although generally mild and self-limited, the resulting haemolytic burden has the potential to increase the risk of infection, graft failure and death. The phenomenon is observed in the transplantation of any solid organ with lymphoid tissue, including the liver. We present a structured case report of passenger lymphocyte syndrome following minor ABO-mismatched liver transplantation, which was initially complicated by blood loss anaemia early in the postoperative period. By reviewing the limited literature of this disorder following liver transplantation, we emphasise common clinical findings and treatment strategies as well as introduce chimerism analysis to confirm resolution.

Severe insulin resistance in a patient with diabetes after treatment with brentuximab vedotin.

A man in his late 60s with a history of well-controlled type 2 diabetes and hepatic cirrhosis presented to the emergency department due to uncontrollable hyperglycaemia following the initial brentuximab vedotin (BV) infusion. BV was initiated as a treatment for mycosis fungoides, a form of cutaneous T-cell lymphoma. The patient was diagnosed with severe hyperglycaemia with ketosis. Empiric treatment with amoxicillin-clavulanic acid, hydration and intravenous insulin infusion was initiated. Hyperglycaemia persisted despite receiving massive amounts of insulin and was corrected only after treatment with high-dose methylprednisolone for suspected type B insulin resistance. Extremely high and difficult-to-treat hyperglycaemia is a rare side effect of BV. Unfortunately, the patient died of upper gastrointestinal bleeding 22 days after discharge. In patients with obesity and/or diabetes mellitus, the blood glucose levels should be carefully monitored when treated with BV.

Steroid-induced psychosis: a spectrum of neuropsychiatric glucocorticoid side effects.

Glucocorticoid-induced neuropsychiatric side effects have been known since their initial usage and frequently manifest in clinical settings. Despite this, they remain unpredictable, variable and complex to manage, impacting patient outcomes and the healthcare system.We report a case of glucocorticoid-induced psychosis after the administration of dexamethasone post-neurosurgical intervention and its evolution with the initiation of chemotherapy. Although initially manic symptoms were prominent, with the beginning of chemotherapy psychotic symptoms dominated the clinical presentation, followed by depressive symptoms. Despite challenges in diagnosis and management, including adverse reactions to antipsychotic treatment, this case provides critical insights into the variable and dynamic nature of neuropsychiatric side effects induced by glucocorticoids.

Challenging diagnosis of IgM multiple myeloma.

IgM monoclonal gammopathies such as IgM myeloma and Waldenström macroglobulinaemia are distinct haematological conditions; however, differentiating between these entities can often present as a challenge.In this review, we explore the challenging diagnosis and treatment of IgM myeloma in a patient presenting with unexplained macrocytic anaemia, elevated serum protein and IgM levels in the absence of t(11;14) and lytic bone lesions that are classically associated with the diagnosis of IgM myeloma. The diagnosis was established based on 40% monoclonal plasma cell population on a bone marrow biopsy, gain of 1q21 on fluorescence in situ hybridisation, cyclin D1 positivity and absence of MYD88 mutation.

Pseudo-thrombotic microangiopathy due to folate deficiency.

Classically, deficiencies of vitamin B12 and folate are associated with megaloblastic anaemia. Additionally, vitamin B12 is able to cause a haemolytic anaemia in the form of pseudo-thrombotic microangiopathy (pseudo-TMA). Here, we present a case of a middle-aged woman with a history of Roux-en-Y gastric bypass who presented with dyspnoea and fatigue and was found to have thrombocytopenia and a non-immune haemolytic anaemia. Work-up for haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, paroxysmal nocturnal haemoglobinuria, infection, malignancy and autoimmune conditions was unremarkable. Her haemolytic anaemia and thrombocytopenia resolved with folate replenishment. She was diagnosed as likely having pseudo-TMA secondary to folate deficiency.

Acute onset facial oedema: an unusual presentation of acute lymphoblastic leukaemia in a child.

A previously fit and well girl of middle-childhood age presented to her local emergency department after waking with upper-facial swelling. She had a 24-hour preceding history of mild vomiting illness and fatigue. Examination revealed palpable splenomegaly and mild cervical lymphadenopathy, with pitting oedema of the forehead, nasal bridge and eyelids. Admission full blood count showed anaemia and neutropenia, and further investigations confirmed a diagnosis of acute lymphoblastic B-cell leukaemia. X-ray of the chest and CT imaging did not reveal any local facial mass, veno-occlusive disease or mediastinal pathology to explain her facial swelling. She was referred to the tertiary paediatric oncology service for commencement of induction chemotherapy.

Metastatic breast cancer and monoclonal gammopathy of undetermined significance resembling multiple myeloma.

Radiological evidence of lytic bone lesions has a wide differential diagnosis including metastatic bone disease, multiple myeloma, primary bone cancers and infection. Here, we present the case of a woman in her 80s found to have lytic bone lesions associated with an IgA paraproteinaemia who was thus presumed to have a diagnosis of multiple myeloma. However, bone marrow biopsy results were indicative of monoclonal gammopathy of undetermined significance. She was subsequently referred to the 'carcinoma of unknown primary (CUP) team', and ultimately diagnosed with metastatic breast cancer following a repeat trephine biopsy. A range of conditions may present with lytic bone lesions and these differentials must be suitably investigated even in the presence of paraproteinaemia. Early CUP team involvement is pivotal to ensure appropriate clinical management and patient support.

Dual pathology: paraneoplastic sarcoidosis emerging at the time of progression from smouldering to symptomatic myeloma.

Sarcoidosis is a systemic disease of unknown aetiology, which is diagnosed based on the presence of non-caseating granulomas on histology. The occurrence of sarcoidosis or a sarcoidosis-like reaction with malignancy has been recognised for several years. Although it has been established that there is an increased risk of lymphoproliferative disorder with sarcoidosis, the association between multiple myeloma and sarcoidosis has rarely been reported. Here, we report the case of woman in her mid-50s with an established diagnosis of smouldering myeloma, who presented with gradually worsening shortness of breath and fatigue after 15 months of active observation. A CT scan of her thorax showed mediastinal lymphadenopathy and the nodes were metabolically active on positron emission tomography CT scan. Endobronchial ultrasound with transbronchial needle aspiration confirmed the diagnosis of sarcoidosis. Further evaluation showed preserved lung function on spirometry. Blood analysis showed a simultaneous rise in the serum lambda-free light chain level from 377 mg/L at initial diagnosis up to 807 mg/L with the kappa/lambda ratio falling to 0.012. Repeat bone marrow aspirate and trephine biopsy showed a 15%-20% infiltrate of lambda light chain-restricted plasma cells with aberrant cyclin D1 expression and abundant sarcoid-like non-necrotising giant cell granulomata. Thus, a diagnosis of paraneoplastic sarcoidosis was established.

Rare case of primary bone lymphoma of the femur.

Primary bone lymphoma is a rare type of lymphoma that arises from skeletal tissue. Most cases described are non-Hodgkin's lymphoma with diffuse large B-cell lymphoma being the most common subtype. While it is common for non-Hodgkin's lymphoma to have secondary skeletal system involvement, primary involvement of the skeleton is surprisingly rare. Primary bone lymphoma accounts for less than 5% of all primary bone malignancies, 4%-5% of extranodal lymphomas and less than 1% of all non-Hodgkin's lymphoma. We present an interesting case of a young adult male who was diagnosed with primary bone lymphoma of the femur. Interestingly, his initial X-ray imaging revealed no osseous abnormality and subsequent MRI revealed an infiltrating mass. The patient was treated with chemotherapy of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone resulting in complete remission.

PET-positive suture granuloma of abdominal wall 56 years post-appendicectomy mimicking recurrence of lymphoma.

We present a unique case of a positron emission tomography (PET)-positive suture granuloma deep to an appendicectomy abdominal wall scar from 56 years prior in a patient with treated lymphoma. The lesion was first detected 8 years ago on a PET scan for new diagnosis of follicular lymphoma, with stable appearances 6 and 7 years later at follow-up. Ultrasound-guided biopsy and flow cytometry of the specimen could not exclude an untreated or recurrent lymphoma; thus, the patient underwent resection of the right iliac fossa abdominal wall lesion. Histopathology results noted granulomatous inflammation surrounding foreign material. The patient had an uneventful postoperative recovery and was discharged from surgical services. In this paper, we review the current literature and discuss the dilemma involved in the diagnosis and management of suture granulomas.