Intravascular large B-cell lymphoma with bone marrow involvement at presentation and haemophagocytic syndrome: two Western cases in favour of a specific variant

AIMS: To report two cases of an unusual form of intravascular lymphoma, characterized by bone marrow involvement at presentation with haemophagocytic syndrome. METHODS AND RESULTS: We describe the clinicopathological features of two patients with intravascular lymphoma primarily involving bone marrow. Both patients complained only of fever with pancytopenia and reactive haemophagocytic syndrome. Diagnosis was made on bone marrow examination, which showed large tumour cells of B-cell lineage confined within the lumen of sinuses. CONCLUSION: These two cases and five previous reports could represent a variant of intravascular lymphoma, characterized by early involvement of bone marrow without dissemination to other organs. This form of intravascular lymphoma, called IVL-HS, seems to be an 'Asian' variant with a high prevalence in Asian people and a very low prevalence in Western countries. At a practical level, bone marrow biopsy may be useful in the diagnosis of intravascular lymphoma when the clinical presentation is restricted to fever of unknown origin with a reactive haemophagocytic syndrome.     

Intravascular lymphomatosis: a clinicopathological study of two cases presenting as an interstitial lung disease

 

Aims:

Intravascular lymphomatosis is an uncommon type of non-Hodgkin's lymphoma characterized by intravascular proliferation of neoplastic lymphoid cells. Although the tumour is basically a systemic disease, eventually involving multiple organs, primary presentation in the lung is rare.  

Methods and results:

We describe the clinicopathological features of two patients with intravascular lymphomatosis presenting in the lung. One patient complained of fever, headache and chest pain; the other, of dyspnoea on exertion and headache. Both patients showed reticulonodular density on chest radiography and decreased diffusion capacity. Lung biopsy showed features characteristic of intravascular lymphomatosis. Malignant lymphoid cells were CD30 positive T-cells of anaplastic large cell type in one patient and B-cells of large cell type in the other. There was a poor response to chemotherapy and both patients died of the disease within 3 months of diagnosis.  

Conclusions:

These cases and 10 previous reports illustrate the need to include intravascular lymphomatosis in the differential diagnosis of interstitial lung disease.     

Detection of circulating tumor cells in a patient with intravascular lymphomatosis: A case study examined by the cytology method

Detection of circulating tumor cells in a patient with Intravascular lymphomatosis (IVL) of the lung is reported. Peripheral blood ceils were suspended in an isotonic sodium chloride solution, and red blood cells were lysed. The suspension was smeared on glass slides, fixed In 95% ethanol solution and stained using the Papanicolaou method. At the diagnosis of IVL, approximately 65 tumor cells were detected in one smear preparation, which contained about 8 × 10⁵ nucleated cells. However, tumor cells disappeared from peripheral blood when those in the lung were eliminated by multiagent chemotherapy. Cytology examination of peripheral blood cells may be of assistance in the early diagnosis of IVL and In the assessment of therapeutic intervention.     

Fatal haemophagocytic syndrome and hepatitis associated with visceral leishmaniasis

Haemophagocytic syndrome (HPS) secondary to infections occurs due to excessive, non-malignant proliferation of histiocytes, with resultant haemophagocytosis. The syndrome is essentially treatable, provided timely etiological diagnosis is achieved. In this report, we present a rare case of a child who hailed from Uttaranchal and presented with severe hepatitis. Bone marrow examination revealed an unexpected diagnosis of HPS secondary to visceral leishmaniasis. Despite initiating appropriate antileishmanial treatment, the child had a fatal outcome.     

Intravascular large B-cell lymphoma presenting with mass lesions in the central nervous system: a report of five cases

We have encountered five cases of intravascular large B-cell lymphoma (IVL) presenting with central nervous system (CNS) mass lesions during their clinical course. The age of the patients ranged from 50 to 74 years and three patients were male. All of these cases histopathologically showed typical intravascular localization of the neoplastic cells in the initial biopsy specimens obtained from sites other than the CNS. Despite multiagent chemotherapy, patients suffered from single or multiple CNS mass lesions 5-44 months after the initial diagnosis of IVL, except for one case in which IVL and the CNS mass lesion were diagnosed at the same time. The subsequent biopsy and autopsy specimens obtained from the CNS mass lesions revealed diffuse infiltration of the tumor cells with perivascular spreading, but minimal or no intravascular components. Immunohistochemical analysis of intravascular tumor cells and CNS mass lesions revealed expression of CD20, CD79a, bcl-2 and negative for CD3e and Epstein-Barr virus encoded RNA. The overall features of the CNS mass lesions were very similar to or indistinguishable from those of the primary CNS lymphomas. This implies that CNS mass lesions in the IVL cases can be correctly diagnosed only by careful attention to clinical and pathological findings. Moreover, there is the possibility that some cases previously diagnosed as primary CNS lymphomas may have include IVL cases. Further investigation is needed to explore this unusual phenomenon.     

Fever of unknown origin characterized by acute kidney injury and nephrotic syndrome diagnosed as intravascular large B-cell lymphoma of kidney: case report and literature review

A 60-year-old Chinese female patient was admitted to the hospital with complaint of intermittent fever for more than seven months. The main clinical manifestations were acute kidney injury and nephrotic syndrome which developed into a hemophagocytic syndrome. The symptoms did not improve with antibiotics. Moreover, prednisone could only reduce the fever. Finally, a kidney biopsy showed many CD20-positive cells in the glomerulus and some in the peritubular capillaries. This led to a diagnosis of renal intravascular large B-cell lymphoma.     

Clinicopathologic characteristics and steroid response of IgM nephropathy in children presenting with idiopathic nephrotic syndrome

There is no detailed information on clinical and immunopathologic features of immunoglobulin M nephropathy (IgMN) in children with idiopathic nephrotic syndrome (INS) in Pakistan. We reviewed our native renal biopsies over 15 years (July 1995-July 2010) and identified 135 cases of IgMN in nephrotic children (≤17 years). Their demographic, clinical and immunopathologic data were retrieved from biopsy reports and case notes. Mean age of this cohort was 7.6 ± 4.2 years. Males were 92 (68.1%) and females were 43 (31.9%). Steroid-dependent NS was seen in 88 (65.2%) cases and steroid-resistant NS in 47 (34.2%). Hematuria was found in 42 cases (31.2%) and hypertension in 27 (19.5%). The most common morphologic change was glomerular mesangial proliferation, found in 89 (65.9%) biopsies. Minor changes were seen in 46 (34.1%) cases and focal segmental glomerulosclerosis (FSGS) in 37 (27.4%). Immunofluorescence microscopy showed diffuse mesangial positivity of IgM in all cases. C3 and C1q were found in 72 (53.3%) and 40 (29.7%) cases, respectively. Our results show that IgMN is a fairly common cause of INS in children in Pakistan. It shows a spectrum of morphologic changes ranging from minor changes to FSGS.     

Plasmablastic lymphoma involving breast: a case diagnosed by fine-needle aspiration and core needle biopsy

Plasmablastic lymphoma (PBL) is a rare lymphoma originating from B-cells with terminal differentiation. Most common anatomic site involved by PBL is the oral cavity. Involvement of other body sites has only rarely been reported. Herein, we report a rare case of EBV-negative PBL involving the breast of an HIV positive 47-year-old woman. The patient presented with decreased vision and photophobia. During physical examination, she was found to have bilateral breast masses and multiple lymphadenopathy. Fine-needle aspiration of one of the breast masses showed large malignant cells with plasmacytoid features. Immunohistochemical studies performed on the core biopsy showed that the tumor cells were positive for common leukocyte antigen CD45 and plasma cell marker CD138, but negative for the pan-B cell markers CD20 and CD79a. Molecular genetic studies showed clonal rearrangement of the immunoglobulin kappa light chain gene. This is the first case of PBL involving the breast reported in English cytological literature.     

Genetic diagnosis and renal biopsy findings in the setting of a renal genetics clinic

As genetic testing becomes more available, its utilization as an early diagnostic tool in nephrology is more common. The objective of the study is to examine diagnostic agreement between the renal biopsy findings and genetic diagnoses. A retrospective study was conducted in February 2022. A total of 28 patients had both genetic diagnosis and histologic results (n = 1 nephrectomy, n = 27 biopsy). We collected clinical, renal biopsy findings, and genetic information. The relationship between the histologic findings and the genetic diagnoses was classified as: concordant, nonspecific, and discordant. A total of 15 males and 13 females were included (mean age = 9.6 years). Clinical suspicion of Alport syndrome was the most common reason for referral (n = 11, 39.3%), followed by nephrotic syndrome (n = 8, 28.5%), “other” (n = 6, 21.4%), cystic kidney disease (n = 1, 3.6%), isolated hematuria (n = 1, 3.6%), and non-nephrotic proteinuria (n = 1, 3.6%). The overall concordance rate between renal histologic and genetic diagnoses was 71.4% (20/28), nonspecific biopsy results were observed in 17.9% (5/28), and discordant results were observed in 10.7% (3/28). All patients referred for suspected Alport Syndrome had pathogenic/likely pathogenic variants in one of the COL4A genes. Two cases of Lowe syndrome and one of PAX2-associated nephropathy had discordant histology findings. Agreement between renal histologic findings and genetic results varies based on the reason for referral. There was a complete agreement for patients referred for Alport Syndrome; However, there were examples that renal biopsy showed secondary findings that were not specifically associated with the underlying genetic results.     

Persistent renal damage following pre-eclampsia: a renal biopsy study of 13 patients

Renal biopsy material was examined from 13 patients who had a history of pre-eclampsia and who were biopsied after delivery. The characteristic 'endotheliosis' associated with pre-eclampsia appeared to resolve at varying rates and was not seen in biopsies taken more than 1 month after delivery. Four of the biopsies showed residual focal segmental glomerular lesions which are described. Focal global glomerular sclerosis (eight biopsies), focal interstitial scarring (seven biopsies) and arteriolo-sclerosis (nine biopsies) were also identified. These findings support those earlier reports which indicated that pre-eclampsia is capable of producing persistent renal damage in a small but significant percentage of cases.     

Isolated adrenal cryptococcosis,diagnosed by fine‐needle aspiration biopsy: A case report

This report documents a case of isolated adrenal gland cryptococcosis without the often reported component of concomitant meningitis or Addison's disease in an immune competent patient. Furthermore, both the patient's lung cancer and adrenal infection with cryptococcus were incidentally discovered during work‐up for syncope in the setting of hyponatremia. This case also underscores the diagnostic value of fine‐needle aspiration biopsy in this unusual presentation. Diagn. Cytopathol. 2014;42:899–901. © 2014 Wiley Periodicals, Inc.     

Ovarian hyperstimulation syndrome and its effect on renal function in a renal transplant patient undergoing IVF treatment: case report

Ovarian hyperstimulation syndrome (OHSS) in a renal transplant patient undergoing assisted conception treatment is reported. A couple with infertility secondary to tubal blockage and pelvic endometriosis received IVF treatment. Ovarian enlargement secondary to OHSS resulted in obstruction in the transplanted kidney and deterioration of renal function. No other systemic manifestations of OHSS were evident. Conservative management was successful and a twin live birth was later achieved by replacement of two frozen-thawed embryos.     

Phosphaturic mesenchymal tumor diagnosed by fine-needle aspiration and core biopsy: a case report and review of literature

Oncogenic (tumor-induced) osteomalacia is a rare paraneoplastic syndrome of phosphate wasting that is frequently associated with phosphaturic mesenchymal tumor (PMT). As the cytologic features of this tumor apparently have not been reported, we describe the fine-needle aspiration (FNA) findings for PMT that arose from the gluteal soft tissue in a patient with hypophosphatemia and multiple fractures secondary to osteomalacia. Smears from the computerized tomography (CT)-guided FNA showed groups of spindle cells having elongated nuclei, fine to moderately coarsely granular chromatin, inconspicuous nucleoli, and delicate cytoplasm. Marked nuclear atypia, mitotic figures, and necrosis were absent. The differential diagnosis included a variety of benign and malignant spindle cell neoplasms such as monophasic synovial sarcoma, leiomyoma, peripheral nerve sheath tumor, fibrosarcoma, and, less likely, metastatic melanoma and sarcomatoid carcinoma. The bland-appearing cytologic features of a spindle cell tumor in a patient with osteomalacia should suggest the diagnosis of PMT.     

Percutaneous renal biopsy of native kidneys: efficiency,safety and risk factors associated with major complications

Introduction

The use of an automated biopsy device and real-time ultrasound (current technology) for percutaneous renal biopsies (PRBs) has improved the likelihood of obtaining adequate tissue for diagnosis and has reduced the complications associated with renal biopsies. Our objective was to evaluate the efficacy and safety of the current PRB procedure and identify possible risk factors for the development of major complications.

Material and methods

We collected all native kidney PRBs performed with current technology in our institute from January 1998 to April 2008. Studied variables were collected from the patient''s chart at the time of the biopsy.

Results

We analyzed 623 (96.4%) of 646 renal biopsies performed with the current automated procedure guided by real-time ultrasound. Although the effectiveness was 97.6%, there were 110 complications. Fourteen (2.24%) of these complications were major: 9 cases of renal hematoma, 2 cases with macroscopic hematuria (which needed blood transfusion), 1 case of intestinal perforation (which required exploratory laparotomy), 1 nephrectomy and 1 case of a dissecting hematoma. The logistic regression analysis demonstrated the following risk factors for developing major complications: diastolic blood pressure ≥ 90 mmHg, RR 7.6 (95% CI 1.35-43); platelet count ≤ 120×10³/µl; RR 7.0 (95% CI 1.9-26.2); and blood urea nitrogen (BUN) ≥ 60 mg/dl, RR 9.27 (95% CI 2.8-30.7).

Conclusions

The observed efficacy and safety of the current technique in the present study were similar to observations in previous studies. Diastolic blood pressure ≥ 90 mmHg, platelets ≤ 120×10³/µl and BUN ≥ 60 mg/dl were independent risk factors for the development of major complications following PRB.     

Case report: metastatic renal carcinoid to the thyroid diagnosed by fine needle aspiration biopsy

We report a case of primary renal carcinoid arising in a horseshoe kidney. To the best of our knowledge this is the first case to be reported in the cytology literature, which has been diagnosed by fine needle aspiration (FNA). A 32-year-old male, presented to the Emory University Hospital, with a renal mass arising in a horseshoe kidney; along with a thyroid mass. FNA of the renal mass resulted in an initial diagnosis of renal cell carcinoma, unclassified. A thyroid aspiration was attempted later, and revealed a neuroendocrine morphology. This was compared with the renal aspiration and both of them were found to have similar morphology. With the help of immunostains, a diagnosis of renal carcinoid tumor metastatic to the thyroid was made. Thus, we demonstrate that renal carcinoid, being a rare entity, can pose a diagnostic challenge.     

Galactosialidosis associated with IgA nephropathy: morphological study of renal biopsy

Galactosialidosis is an autosomal recessive lysosomal disease associated with a deficiency of β-galactosidase and neuraminidase. Described herein is the case of a young adult who had been diagnosed with galactosialidosis at 8 years of age. At the age of 30 years, proteinuria and hematuria appeared and the patient underwent a renal biopsy 1 year later. Light microscopy of the kidney sections indicated fine granular contents in the cytoplasm of glomerular endothelial and epithelial cells, arteriolar smooth muscles and proximal tubular epithelial cells on periodic acid silver–methenamin (PAM) stain. Electron microscopy of these cells indicated enlarged, smooth endoplasmic reticulum and lysosomes containing 150 nm-wide rods with a fine lattice structure at 66 Å periodicity. Moreover, electron-dense deposits were located in the paramesangial area. Immunofluorescence staining indicated diffuse and global anti-human IgA and C3-positive staining as a mesangial pattern. Given these findings this patient was therefore diagnosed with both galactosialidosis and IgA nephropathy. This is the first report to describe light and electron microscopy observations of storage materials in the kidneys in young/adult galactosialidosis.     

Epithelioid hemangioendothelioma: Report of a case diagnosed by fine-needle aspiration

A case of epithelioid hemangioendothelioma suggested by fine-needle aspiration biopsy is reported. The clinical, cytological, histopathological, and postmortem findings are described. The diagnosis was confirmed by cyto-histoimmunochemical studies.     

Evolution of Anelloviridae strains distribution in serial blood and biopsy samples from a renal transplant patient

A few studies investigated the natural history of viruses belonging to the family Anelloviridae in transplant patient. The case of a 64-year-old kidney transplant recipient is described. Molecular analysis of serial blood samples collected before and after transplant was performed during a period of 510 days. Two kidney biopsies were also analyzed. All blood samples tested positive for Anelloviridae DNA, with the identification of sequences belonging to the three taxonomic genera identified in humans. Sequences distribution during the follow-up was multimodal. A sequence nearly identical to one present in the blood before transplant was further characterized in one biopsy sample.