An Intriguing Case of Amyloidosis Leading to PAGE Kidney in a Post Renal Transplant Recipient: A Case Report.

Amyloidosis is an infiltrative disease where amyloid fibrils get deposited in the organs like kidney, liver and spleen. Amyloid deposition in the kidneys classically meant deposition in the glomeruli and mesangium until 2008 when interstitial amyloid deposits were isolated and named as` Leukocyte cell-derived chemotaxin 2-associated amyloidosis. It is a progressive disease which clinically manifests as slowly progressive renal dysfunction and/or proteinuria. Our case 34 year old renal transplant recipient underwent graft biopsy post transplantation which revealed interstitial LECT-2 amyloid deposits. Unfortunately, he developed page kidney post biopsy which was managed conservatively with percutaneous drainage. Supplementary Information: The online version contains supplementary material available at 10.1007/s12291-022-01072-6.

Adenosquamous carcinoma lung radiologically mimicking pneumonia: A potentially disastrous diagnostic challenge in an unusual malignancy.

Lung cancer is among the most frequently diagnosed cancers and the world's leading cause of cancer-related death. Radiology remains the mainstay for timely diagnosis; however, atypical radiologic patterns are known, and these may be misdiagnosed as infectious or inflammatory pathology, particularly in the absence of smoking history. We report herein an account of an older male nonsmoker who presented radiologically with bilateral diffuse pulmonary infiltrates, simulating pneumonia, but was eventually diagnosed with adenosquamous lung carcinoma. The delay in diagnosis and subsequent unfortunate rapid deterioration of our patient serves as a reminder for clinicians to consider lung cancer in patients with clinical/radiologic findings suggestive of pneumonia, especially in nonsmokers or cases refractory to antibiotic therapy.

Primary neurolymphomatosis presenting as foot drop: A rare case report.

ABSTRACT: Neurolymphomatosis (NL) is the direct infiltration of the peripheral nervous system (PNS) by lymphoma cells and represents the least common form of PNS involvement by lymphoma. Clinical presentation is varied, and early diagnosis remains challenging. Nerve biopsy remains the diagnostic gold standard, use of magnetic resonance imaging (MRI) and fluorodeoxyglucose-positron emission tomography (FDG-PET) may help in diagnosis and selecting targets for biopsy. We report an account of an older male patient who presented with subacute onset multifocal neuropathy involving bilateral lower limbs and left foot drop. The patient was initially misdiagnosed as chronic inflammatory demyelinating polyneuropathy; however radiological assessment revealed fusiform thickening of the L5 nerve root and biopsy from the site revealed extensive infiltration by diffuse large B cell type non-Hodgkin lymphoma (NHL). High index of suspicion is vital to ensure correct diagnosis, timely treatment, and to improve patient survival.

Diagnostic Dilemma: Cardiorenal Syndrome As an Unusual Presentation of IgG4-Related Disease.

IgG4-related kidney disease (IgG4 RKD) is a rare clinical entity characterized by lymphoplasmacytic infiltration rich in IgG4-positive plasma cells along with fibrosis affecting several organs. Tubulointerstitial nephritis is commonly the predominant finding on kidney biopsy. Our patient was admitted with a provisional diagnosis of cardiorenal syndrome of unknown etiology. The patient was dialysis dependent for around 45 days following which kidney biopsy revealed features of acute tubulointerstitial nephritis (ATIN) with IgG4-positive plasma cells and no glomerular involvement. Positron emission tomography-computed tomography was supportive of findings of sialadenitis along with myocarditis. Our patient responded to treatment with steroids with definitive improvement in both renal and cardiac functions. This case highlights the importance of IgG4 RKD as an important differential in patients with ATIN presenting as a clinical syndrome.

Radiation Induced Liver Injury: Collateral Damage Radiologically Simulating Interval Metastasis in Carcinoma Esophagus; a Diagnostic Dilemma Resolved Through Liver Biopsy.

F-18-fluorodeoxyglucose positron emission tomography (18-FDG PET/CT) is increasingly being used in patients with cancer, both for baseline staging and for evaluation of treatment response. However, in patients with incidental irradiation of the liver during radiotherapy, particularly for lower gastrointestinal tract cancers, increased focal F-18-fluorodeoxyglucose positron emission tomography avidity may be the result of collateral radiation induced liver damage rather than metastases. Awareness of this pathologic entity and correlation with with other imaging, clinical and laboratory findings including liver biopsy is vital to avoid misinterpretation and overstaging of the carcinoma in these patients. We encountered such a scenario in an elderly female patient with distal esophageal squamous cell carcinoma patient, who developed F-18-fluorodeoxyglucose positron emission tomography avid left lobe liver lesion post neoadjuvant radiotherapy, simulating interval metastasis. A liver biopsy ruled out malignancy and helped to clinch the correct diagnosis of radiation induced liver injury.

Hodgkin Variant of Richter's Transformation in Chronic Lymphocytic Leukemia (CLL): An Illustrative Case Report and Literature Review.

Hodgkin lymphoma variant of Richter's transformation (HL-RT) is a rare event, occurring in < 1% chronic lymphocytic leukemia (CLL) cases, of which, in < 10% cases, HL is the first finding leading to a diagnosis of CLL that co-exists simultaneously. Here we report a 60 years old male patient who presented with an outside diagnosis of lymphocyte-rich classical HL. On evaluation, he had only B-symptoms in the form of low-grade fever and weight loss. Peripheral smear revealed mild leukocytosis with an absolute lymphocytosis and a few smudge cells. Bone marrow (BM) aspirate and biopsy exhibited diffuse infiltration by a small cell, low grade, Non-Hodgkin's lymphoma with no immunohistochemical evidence of HL. Flow cytometry performed on BM was consistent with classical immunoprofile of CLL. Meanwhile the lymph node received for review revealed diffuse effacement of nodal architecture by small mature lymphocytes with immunoprofile of CLL expressing CD20, CD5, and CD23. Interspersed between these cells, were a few eosinophils along with classical Reed Sternberg cells, expressing CD30, MUM-1, CD15, and dim PAX-5, with a surrounding rosette of T-Cells highlighted by CD3 and PD-1 and negative for CD45, CD20, and EBV immunohistochemistry. Fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed hepatosplenomegaly with multiple supra/infra diaphragmatic lymph nodes. So, a final diagnosis of HL-RT in CLL was considered. The patient is currently doing well after the first cycle of ABVD chemotherapy. HL-RT occurring in CLL is a rare event with heterogeneous clinical presentation, morphology, clonal origin, disease course, prognostic features, and survival.

Rare, Yet Emerging Cause of Graft Dysfunction-ALECT 2 Amyloidosis.

Amyloidosis is characterized by pathological deposition of abnormal protein aggregates in various tissues, AL protein being the commonest. Amyloidosis derived from leukocyte cell-derived chemotaxin 2 (LECT2) is a recently recognized form of amyloidosis in the United States with predominant involvement of kidney and liver. We present a case of ALECT2 renal amyloid in a transplant recipient who presented with gradual worsening of graft function and subnephrotic proteinuria. To our knowledge, this is first case of LECT2 amyloidosis from Northern India in a transplant recipient. There is no effective therapy for amyloidosis derived from leukocyte cell-derived chemotaxin 2.

Solitary fibrous tumor of the ovary.

Solitary fibrous tumors commonly occur in the pleura and are rare elsewhere, especially in the female genital system. We present a case of a solitary fibrous tumor arising from the ovary in a young female in the reproductive age group. The tumor could be excised laparoscopically. We also describe the histopathological and immunohistochemical features that can help establish its diagnosis.

A novel CFHR1-CFHR5 hybrid leads to a familial dominant C3 glomerulopathy.

The intrinsic similarity shared between the members of the complement factor H family, which comprises complement factor H and five complement factor H-related (CFHR) genes, leads to various recombination events. In turn these events lead to deletions of some genes or abnormal proteins, which are found in patients with atypical hemolytic uremic syndrome or C3 glomerulopathies. Here we describe a novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein. This deletion was found in four family members presenting with a familial dominant glomerulopathy histologically classified as an overlap of dense deposit disease and C3 glomerulonephritis. Affected patients exhibited permanently low C3 and factor B levels and high amounts of activation fragments sC5b9 and Bb, indicating a systemic alternative pathway dysregulation. The abnormal protein, characterized by Western blot and immunoprecipitation, was shown to circulate in association with CFHR1 and CFHR2, attributable to its two N-terminal dimerization motifs. The presence of this protein is associated with a perturbation of Factor H activity on the C3 convertase decay. Thus, our study highlights the role of CFHRs in the physiopathology of C3 glomerulopathies and stresses the importance of screening CFHRs in all familial C3 glomerulopathies. Such hybrids described till now were always associated with familial forms.

Lymphovascular Malformation - A Report of Two Cases.

Congenital Vascular Malformations (CVM) represents a group of vascular anomalies that are the result of defective development of the vascular system. Lymphatic malformation consists of dilated lymphatic vessels caused by occlusion of the lymphatic drainage system due to congenital malformations or acquired causes such as the effects of trauma, infection, or surgery. Lymphaticovenous Malformation (LVM) is composed of lymphatic and venous channels. The most common sites are the neck and axilla. We report two cases of a four and a half-year-old and a seven-year-old child who presented with complaints of swelling in arm and groin respectively. A provisional diagnosis was made on radiological investigations. Excision was done and the diagnosis was confirmed on histopathology and immunohistochemistry. The cases which were presented as LVMs are rare congenital tumours and require a high index of suspicion on part of pathologist and surgeon to reach a definitive diagnosis.

Gastrointestinal Histoplasmosis: A Case Series.

Histoplasmosis is an invasive mycosis caused by inhalation of the spores of dimorphic fungi Histoplasma capsulatum. The disease manifests in the lung as acute or chronic pulmonary histoplasmosis and in severe cases gets disseminated in multiple organs like skin, adrenal gland, central nervous system, lymph node, liver, spleen, bone marrow, and gastrointestinal tract. It occurs most commonly in immunodeficient patients like HIV-positive patients and transplant recipients, while immunocompetent hosts are affected rarely. In cases of gastrointestinal histoplasmosis, the samples are collected for culture and biopsy should be sent for histopathological examination for definitive diagnosis. We conducted a retrospective study of colonic biopsies performed in the department of gastroenterology in a tertiary care hospital of north India from January 2014 to December 2015. Five cases of colonic histoplasmosis were diagnosed on histopathology out of which 4 patients were from north India while 1 patient was from Myanmar. The patients presented with various complaints, including loose stools, diarrhea, altered bowel habits, and gastrointestinal bleeding. The prognosis is very good after early and aggressive treatment while the disease is fatal if it remains untreated. In our study, 2 patients died within few days of diagnosis due to delay in the diagnosis, dissemination, and associated complications. Other patients were started on amphotericin B deoxycholate and are under follow-up. An early diagnosis of gastrointestinal histoplasmosis is important as appropriate treatment leads to long-term survival while untreated cases are almost fatal.

Bilateral Tonsillar Enlargement as a First Manifestation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with an Unusual Interfollicular Pattern of Infiltration.

Tonsillar lymphoma usually presents as unilateral or bilateral enlargement or as an ulcerative or fungating lesions. Most lymphomas that involve the tonsils are diffuse large B-cell lymphomas and primary low grade lymphomas are exceptional. We report a case of primary B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) affecting tonsils with interfollicular pattern of infiltration in a 54-year-old female who clinically presented with bilateral tonsillar enlargement, sore throat, dysphagia and respiratory difficulty, unresponsive to the conservative treatment. To the best of our knowledge, till date only six cases of CLL/SLL infiltrating tonsils have been reported in the English literature, three of these were known cases of CLL/SLL prior to tonsillectomy. In the present case diagnosis of CLL/SLL was first time established on tonsillar histomorphology and that too with an unusual interfollicular pattern of infiltration.

Coexistent Nodal Diffuse Large B-Cell Lymphoma With Extrapulmonary Tuberculosis: A Rare Case.

Extrapulmonary tuberculosis coexistent with lymphomas in the same organ are rare and have been reported in the literature. The most common organs that are involved are small bowel, bronchus, kidney, and lymph nodes. Interestingly, the lymphoma that is commonly present with extrapulmonary tuberculosis is Hodgkin's lymphoma followed by low-grade non-Hodgkin's lymphoma. In the present study, we report a 60-year-old man with complaints of fever, loss of appetite, and generalized weakness. On investigation, generalized lymphadenopathy was noted, and the biopsy of cervical lymph node revealed coexistence of diffuse large B-cell lymphoma with extrapulmonary tuberculosis. This case is the second reported case of diffuse large B-cell lymphoma with extrapulmonary tuberculosis in the world and the first in India.

Cervical embryonal rhabdomyosarcoma and ovarian Sertoli-Leydig cell tumor with congenital absence of unilateral ovary.

We report this first case of synchronous cervical rhabdomyosarcoma and Sertoli-Leydig cell tumor (SLCT) of ovary with congenital absence of unilateral ovary and fallopian tube in a 16-year-old female patient. A review of the English-language literature identified only six cases of double malignancies with cervical rhabdomyosarcoma and SLCT but none of the case was associated with congenital absence of ovary and fallopian tube. When such multiple conditions involving one system occur in an individual, the possibility of single etiology arises, but beside some hypothesis, no definite link could be established in any of the cases reported.