Castleman's disease or angiofollicular lymphoid hyperplasia is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. The disease can present at any extra thoracic site with lymphoid tissue such as retroperitoneal, mesentery, axilla, and pelvis. Hepatic localization castleman disease is very rare in children. Herein, we reported a case of Castleman's disease arising from the lymph node in hilum of liver.
A 5 -year-old girl with chief complaint of abdominal pain for two months which exaggerated in last three days was referred to the hospital. On routine physical examination, only a generalized abdominal pain was noticed. Routine laboratory investigations and Chest X-Ray were normal. Abdominal Sonography revealed a 3.7 × 3.1 cm solid mass in the hilum of the liver. On the MRI images, a lobulated mass in the portal hepatic associated with mass effect on the portal vein was visible. Histological examination revealed expansion of mantle zone in lymphatic nodules accompanied by burnt out germinal centers. This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease. The patient underwent a laparotomy. The patient had an uneventful postoperative course.
This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease.
Castleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman’s disease have been reported in the literature.
A 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected.
Castleman’s disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.
Castleman’s disease; Hyaline vascular variant; Surgery; Unicentric
Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal masses and causes a wide range of clinical symptoms. Histologically, Castleman’s disease is classified as either a hyalinic vascular or plasma cell variant. The prognosis mainly depends on the histological type and broadly varies. We herein report our sonographic findings in a patient with Castleman’s disease, including gray-scale ultrasonography, color Doppler ultrasonography, and sonoelastography ultrasonography, which have not been previously reported in the literature. These findings allowed for a preoperative diagnosis and avoidance of overly aggressive therapy.
A 28-year-old European female patient with unicentric Castleman’s disease of hyalinic vascular type (HV) restricted to the axilla was referred to us because of a 4-month history of a painless, solitary mass located in the left axilla. The patient’s medical history was unremarkable.
Castleman’s disease is a pathologic entity of unknown etiology and pathogenesis. In this case report of unicentric HV-type CD, we demonstrate that typical sonographic findings can lead to a preoperative diagnosis of Castleman’s disease. Core needle biopsy usually allows for a final diagnosis and helps to avoid unnecessary operations and overtreatment.
Castleman’s disease; Giant lymph node hyperplasia; Ultrasonography; Core needle biopsy
Castleman's disease, or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). Two clinical entities have been described: a unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. Also, three histopathological subtypes have been described: hyaline-vascular, plasma cell, and a mixed variant. Preoperative diagnosis of hyaline-vascular Castleman's disease is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy is the complete surgical excision.
Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown etiology. Clinically, it occurs as a localized (unicentric) disease or as a systemic (multicentric) disease. Unicentric Castleman’s disease (UCD) presents as a solitary mass and primarily affects the mediastinal, retroperitoneal, and cervical lymph nodes. In contrast to multicentric CD, which involves peripheral lymphadenopathy and numerous systemic symptoms, UCD is not typically associated with generalized symptoms. Three main distinct histologic variants are recognized: hyaline-vascular type, plasma cell type, and mixed type. Extranodal CD is rare. Specifically, UCD exclusively in the spleen is extremely rare, with only 2 cases described in the literature to date. Here, we describe an asymptomatic 75-year-old man with a 5.7 cm × 4.5 cm sized heterogenous enhanced mass located in the spleen. He underwent surgical resection for diagnosis and treatment. A pathologic examination indicated the hyaline-vascular type of CD. In this patient, the preoperative diagnosis was difficult to determine, and therefore, invasive procedures were required.
Castleman’s disease; Hyaline-vascular type; Spleen
Three histological variants (hyaline vascular, plasma cell, and mixed) and two clinical types (localized and multicentric) of Castleman's disease have been described. The risk of progression to lymphoma is higher in multicentric Castleman's disease and is associated with poorer outcomes and higher mortality rate. Multicentric Castleman's disease often requires systemic therapy. Complete resection of the involved node in localized Castleman's disease is curative, with no reported recurrences.
We report a case of a 66-year-old female with systemic symptoms and bilateral cervical lymph nodes which were initially diagnosed as the hyaline vascular variant of Castleman's disease and two years later after the initial diagnosis she was confirmed to B cell lymphoma. Following the treatment with radiation therapy to the cervical area and combination chemotherapy complete response was achieved.
Although it has rarely been reported, the malignant potential of the Castleman's disease must be kept in mind.
Castleman's disease is a distinct form of lymph node hyperplasia divided into a solitary and a multicentric type. The solitary type occurs most commonly in the mediastinum and is usually asymptomatic. We present a patient with Castleman's disease of the hyaline-vascular solitary type located in the retroperitoneum.
The patient was a 38-year-old male, who presented to our hospital with fever. The imaging workup revealed a retroperitoneal mass, measuring 4 × 6 cm, located lateral to the aorta, inferior to the left renal artery and vein, and posterior to the left testicular vein. At workup, a solid hepatic lesion, 3 cm in diameter, located in the left lobe of the liver, segment IV, was also identified. Both lesions were surgically excised. The retroperitoneal tumor had the features of angiofollicular hyperplasia (Castleman's disease), hyaline-vascular type, whereas a diagnosis of focal nodular hyperplasia was made for the hepatic lesion. The patient is well at fourty months followup postoperatively. Surgical excision is the treatment of choice for unifocal Castleman's disease.
Castleman’s disease, also known as angiofollicular hyperplasia, is a disease of unsettled complexity and morbidity, usually occurs in the mediastinum and less frequently in the neck as a solitary mass and is often associated with other illnesses. Two variants have been recognized, the hyaline vascular type which is mare common and the less common plasma cell type with a third hybrid or intermediate type that shares features with both. It has been described with AIDS and Kaposi’s sarcoma as well as follicular dendritic cell tumours.
We report a case of Castleman’s disease of the hyaline vascular type arising in the left supra-clavicular region in 41 year old male without any associated systemic illness. The neck is an uncommon location for this rare disease.
Giant lymphnode hyperplasia; Hyalin; Plasma cell
Castleman disease (CD) is a rare benign disorder characterised by hyperplasia of lymphoid tissue that may develop at a single site or throughout the body. The etiology of this disorder is unclear, although the histopathological presentation can be differentiated into a hyaline vascular variant, a plasma cell variant and a mixed variant. Clinically, it has been recorded that 3 manifestations of CD are characterized: a localized unicentric type, a generalized multicentric type and a mixed form. Surgery remains the main treatment for resectable unicentric CD, since removal of the large node is possible without further complications. No consensus has been reached concerning the most adequate treatment for irresectable unicentric CD.
Case report of a 67 year old woman.
This report, describes the case of a 67-year-old woman with unicentric Castleman disease located in the right lower abdomen. The patient had symptoms of fatigue, dyspnoea and pain in the right lower abdomen. Computed tomography (CT)- examination revealed a tumour, which had grown to form a close relationship with the common iliac vessels and the sacral bone. A Laparotomy procedure revealed that the tumour was an irresectable mass. Neo-adjuvant radiotherapy (40 Gy) was administered in order to downsize the tumour. Six weeks later a new CT-scan revealed a major reduction of the tumour, which enabled a successful radical resection of the tumour to be performed. Histopathological analysis of the tumour showed the hyaline vascular type of CD.
Neo-adjuvant radiotherapy should be considered in case of an irresectable unicentric CD.
Castleman’s disease is a rare lymphoproliferative disorder characterised by lymph node follicular hyperplasia with abnormal interfollicular vascular growth. The authors report a female patient presenting with erythematous and pruritic skin lesion. A shave biopsy of the lesion revealed a Clark level III melanoma and sentinel lymph node biopsy revealed a microscopic focus. Staging positron emission tomography scan revealed an abnormal soft tissue mass with increased 18F-fluorodeoxyglucose accumulation in the anterior mediastinum, raising suspicions of malignancy. The patient underwent an elective resection of the mass and histological sections revealed features of Castleman’s disease of the hyaline vascular type. Vascular endothelial growth factor (VEGF) has been associated with the increased vessel density in this condition. VEGF secretion by tumour cells in solid tumours such as melanomas could be a predisposing factor for Castleman’s disease. Future research is needed to find an association between VEGF-secreting solid tumours and the development of lymphoproliferative tumours such as Castleman’s disease.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2872096831190851.
Castleman disease (CD), described as a heterogeneous lymphoproliferative disorder, can be divided into different subtypes according to clinical appearance (unicentric and multicentric form) and histopathological features (hyaline vascular, plasma cell, mixed type, human herpesvirus 8–associated and multicentric not otherwise specified). Unicentric CD is known to be usually of the hyaline vascular variant, plasma cell and mixed type of this form are quite uncommon. Malignancies are mainly associated with the multicentric form. We report a rare case of unicentric mixed variant CD evolving into intrabronchial, extramedullary plasmacytoma.
Intrabronchial mass with consequential obstruction of the left main bronchus, left lung atelectasis and mediastinal lymphadenomegaly was detected by chest CT in our patient suffering from cough and hemoptysis. Pulmonectomy was performed, histopathological and immunhistochemical analysis of lymph nodes revealed mixed type of CD with interfollicular monotypic plasma cell proliferation. The intrabronchial mass consisted of monotypic plasma cells confirming plasmacytoma. Systemic involvement was not confirmed by further tests.
Although malignancies more often present in multicentric CD that usually belongs to the plasma cell subtype, this case confirms the neoplastic potential of the rarest, unicentric mixed variant of CD.
Castleman disease; Unicentric; Mixed variant; Extramedullary plasmacytoma; Intrabronchial plasmacytoma
Castleman’s disease (CD), also called giant lymph nodal hyperplasia, is a lymphoproliferative disorder secondary to lymphoid follicle hyperplasia and marked capillary proliferation with endothelial hyperplasia. It presents as enlarged lymph nodes in the mediastinum, neck, groin, axilla and other sites. On clinical examination as well as gross examination, this disease mimics lymphomas and tuberculosis. Since cytological appearances vary depending on the type and extent of hyperplasia, fine needle aspiration cytology (FNAC) findings may not always be conclusive in all cases. We studied aspirates in two cases of CD, one of which presented with multiple enlarged axillary lymph nodes and the other with enlarged cervical lymph node. Cytology revealed reactive lymphadenitis with hyalinized capillaries and other features. Lymph node excision in both cases confirmed the diagnosis.
Cytology; Castleman’s disease; lymph node; fine needle aspiration cytology
A patient presenting with predominantly gastrointestinal symptoms and a history of myocardial infarction was found to have ascites, hepatosplenomegaly, para-aortic lymphadenopathy, thrombocytosis, and a paraproteinaemia. A jejunal biopsy specimen showed lymphangiectasia and histology of the spleen and lymph nodes showed angiofollicular hyperplasia or Castleman's disease of the hyaline vascular type. This association has not previously been described and, moreover, systemic symptoms are unusual in this variant of Castleman's disease.
Castleman's disease is a rare form of localized lymph node hyperplasia of uncertain etiology. Although the mediastinum is the most common site of involvement, rare cases occurring in lymph node bearing tissue of other localization have been reported, including only a few intramuscular cases. Unicentric and multicentric Castleman's disease are being distinguished, the latter harboring an unfavorable prognosis.
Here, we present a case of unicentric Castleman's disease in a 37-year-old woman without associated neoplastic, autoimmune or infectious diseases. The lesion was located in the femoral region of the right lower extremity and surgically resected after radiographic workup and excisional biopsy examinations. The tumor comprised lymphoid tissue with numerous germinal centers with central fibrosis, onion-skinning and rich interfollicular vascularization. CD23-positive follicular dendritic cells were detected in the germinal centers and numerous CD138-positive plasma cells in interfollicular areas. The diagnosis of mixed cellularity type Castleman's disease was established and the patient recovered well.
In conclusion, the differential diagnosis of Castleman's disease should be considered when evaluating a sharply demarcated, hypervascularized lymphatic tumor located in the extremities. However, the developmental etiology of Castleman's disease remains to be further examined.
Castleman's disease; unicentric; mixed cellularity type; follicular dendritic cells
Castleman disease is a rare lymphoproliferative lesion that is predominantly found in the mediastinum. Retroperitoneal and pararenal localizations are very rare. We describe a 36-year-old man with a hyaline vascular type of Castleman disease involving renal parenchyma and a paraaortic lymph node. Most reported renal Castleman disease was plasma cell type with systemic symptoms. Herein, we report the first Korean case of the hyaline vascular type of Castleman disease involving the renal parenchyma and the paraaortic lymph node simultaneously.
Kidney; Hyaline vascular type, Multicentric; Giant lymph node hyperplasia
Castleman's disease, also known as angiofollicular hyperplasia, usually occurs in the mediastinum and rarely in cervical region as solitary mass. Histopathologically four variants have been recognized (Castleman et al. Cancer 1954;9:822–30) hyaline vascular type—most common type (Keller et al. Cancer 1972;29:670–83) plasma cell type (Salisbury, Pediatric Pathol 1990;10:609–15) transitional type (Shahidi, Mayo Clinic Proc 1995;70:969–77) stromal rich type. We report a case of Castleman's disease of typical histopathological picture of angio follicular hyperplasia arising from the right carotid triangle of neck in 12-year-old child without any associated systemic illness.
Castlman's disease; angio follicular hyperplasia; neck
Castleman’s disease is a rare and poorly understood disease entity that may resemble more common conditions and represents a clinical challenge to the treating surgeon.
In this report, we describe a case of a 61-year-old Caucasian woman with a symptomatic retroperitoneal mass. The specimen obtained from her resection contained a protuberant encapsulated mass, exhibiting microscopic features consistent with localized, unicentric Castleman’s disease. These characteristics included architectural features and immunohistochemical findings consistent with the hyaline vascular variant of Castleman’s disease.
We report a very rare case of a retroperitoneal hyaline vascular type of Castleman’s disease. We discuss the diagnostic dilemma Castleman’s disease may present to the surgeon, with an emphasis on multidisciplinary management of these patients. We also review current data on pathogenesis, treatment and outcomes.
Angiofollicular lymph node hyperplasia; Castleman’s disease; Management; Multidisciplinary care
Castleman disease (CD) is a rare atypical lymphoproliferative disease, pathologically classified as hyaline vascular, plasma cell type and mixed type variant. The underlying cause of CD is unknown, however several theories including autoimmunity have been proposed. We describe a patient diagnosed with unicentric mixed variant CD and Hashimoto thyroiditis, concurrently. She was staged with fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) and the disease was localized to the mediastinum. After 6 cycles of chemotherapy consisting of vincristine and prednisone, the mediastinal lymph nodes regressed, but did not disappear from the CT scan. However, FDG-PET/CT showed complete metabolic response. Although the role of FDG-PET/CT in staging and evaluation of treatment response is controversial, this case shows that PET/CT can be effective and even better for staging and response evaluation. This case is also unique as there no case of CD in association with Hashimoto thyroiditis has been reported previously. However, the possibility of a coincidental association must be raised, especially when the high prevalence of Hashimoto thyroiditis is considered.
Castleman disease; FDG-PET; Hashimoto thyroiditis
Castleman's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck. It very rarely affects the renal sinus. We report a case of Castleman's disease arising in the renal sinus in a 64-year-old man. The patient visited the hospital with the chief complaint of hematuria. Abdominal computed tomography revealed a homogeneous mass in the sinus of the left kidney, radiologically interpreted as a malignant urothelial tumor. Subsequently, nephroureterectomy was performed, after which microscopic examination of the specimen revealed a diffuse lymphoproliferative lesion with reactive lymphoid follicles of various sizes and prominent plasma cell infiltration of interfollicular spaces, highlighted by immunohistochemical staining for CD138. The lesion was diagnosed as Castleman's disease of the plasma cell type. Although preoperative diagnosis of Castleman's disease is difficult and the incidence is exceedingly rare, it should be considered in the differential diagnosis of renal sinus tumors.
Giant lymph node hyperplasia; Plasma cell type; Renal sinus; Urothelial malignancy; Kidney
Clinical, pathological, and viral markers were used to classify patients with Castleman's disease into groups with markedly different outcomes and disease associations. This system could aid in providing physicians and patients with a realistic prognostic outlook.
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Describe the centricity and histopathology profiles of each of the three classes of HIV-negative patients identified in this analysis.Correlate nosological classification and outcomes in patients with Castleman's disease.
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Castleman's disease is a rare primary disease of the lymph nodes with limited available clinical information.
A systematic literature search identified 416 cases amenable to detailed analysis.
In HIV− patients, centricity, pathology type, the presence of symptoms, gender, and age all predict outcome in univariate analyses. The 3-year disease-free survival (DFS) rate for patients with unicentric hyaline vascular disease (49.5% of cases, class I) was 92.5%, versus 45.7% for those with multicentric plasma cell disease (20.2% of cases, class III) and 78.0% for those with any other combination (22.6% of cases, class II) (p < .0001). HIV+ patients (class IV) exclusively presented with multicentric plasma cell disease and had a 3-year DFS rate of only 27.8%. Kaposi's sarcoma and lymphoma were observed in 59.3% and 9.4% of HIV+ patients and in 2.6% and 3.6% of HIV− patients (p < .0001). Paraneoplastic pemphigus and the syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes were observed exclusively in HIV− patients at a rate of 1.3% and 1.8%, respectively.
Clinical, pathological, and viral markers allow for the classification of Castleman's disease into groups with markedly different outcomes and disease associations.
Castleman's disease; Lymph node; HIV; Viral infection
Most of the previously reported cases of Castleman’s disease (CD) in the neck were of the hyaline vascular type and the most common sign was an asymptomatic neck mass. To achieve a more general recognition of CD in the neck, we present four cases of CD in the neck with unusual presentations.
Patients and Methods
We describe four cases of CD in the neck with unusual clinical manifestations, different histopathologic subtypes and discuss the problems arising in their clinical findings.
Two cases were plasma cell type with multiple bilateral enlarged lymph nodes and systemic manifestations. Two cases presented as a solitary mass were hyaline vascular type. One case underwent twice local recurrence and evolved from the hyaline vascular CD to follicular dendritic cell sarcoma.
Cervical plasma cell CD may have a tendency to present as multiple masses on the both sides with systemic manifestations. It is necessary to pay more attention on the overgrowth of follicular dendritic cells in the hyaline vascular CD, which might represent a precursor of follicular dendritic cell sarcoma.
Castleman’s disease; Neck; POEMS syndrome; Follicular dendritic cell sarcoma; Clinical manifestation
Castleman’s disease is a benign lymphoproliferative disease characterised by hyperplasia of lymphoid follicles. It can affect any lymph nodes in the body. Here we describe a caucasian patient who presented with six months history of shortness of breath with CT scan confirming an 8 cm segment of consolidated lung in left hilum. PET scan revealed a mass measuring 68x80x55 mm extending from the left hilum out into the left upper lobe containing area of calcification with SUV max 4.8. The differential diagnosis included atypical sequestration, hamartoma and primary lung malignancy. The patient underwent left video assisted enucleation of the lesion. The histology confirmed the diagnosis of Castleman’s disease.
Benign lung lesion; Castleman’s disease and VATS
A simian homologue of Kaposi's sarcoma–associated herpesvirus (KSHV), the eighth human herpesvirus (HHV8), was isolated from a simian immunodeficiency virus (SIV)-infected rhesus macaque (Macaca mulatta) that developed a multicentric lymphoproliferative disorder (LPD). This simian rhadinovirus is genetically similar to a recently described rhesus rhadinovirus (RRV) (Desrosiers, R.C., V.G. Sasseville, S.C. Czajak, X. Zhang, K.G. Mansfield, A. Kaur, R.P. Johnson, A.A. Lackner, and J.U. Jung. 1997. J. Virol. 71:9764–9769) and is designated RRV 17577. RRV 17577 was experimentally inoculated into rhesus macaques with and without SIVmac239 infection to determine if RRV played a role in development of the LPD observed in the index case. In contrast to control animals inoculated with SIVmac239 or RRV alone, two animals coinfected with SIVmac239 and RRV 17577 developed hyperplastic LPD resembling the multicentric plasma cell variant of Castleman's disease, characterized by persistent angiofollicular lymphadenopathy, hepatomegaly, splenomegaly, and hypergammaglobulinemia. Hypergammaglobulinemia was associated with severe immune-mediated hemolytic anemia in one RRV/SIV-infected macaque. Both RRV/SIV-infected macaques exhibited persistent RRV viremia with little or no RRV-specific antibody response. The macaques inoculated with RRV alone displayed transient viremia followed by a vigorous anti-RRV antibody response and lacked evidence of LPD in peripheral blood and lymph nodes. Infectious RRV and RRV DNA were present in hyperplastic lymphoid tissues of the RRV/SIV-infected macaques, suggesting that lymphoid hyperplasia is associated with the high levels of replication. Thus, experimental RRV 17577 infection of SIV-infected rhesus macaques induces some of the hyperplastic B cell LPDs manifested in AIDS patients coinfected with KSHV.
B cell hyperplasia; simian immunodeficiency virus; rhesus rhadinovirus; lymphoproliferative disorders; nonhuman primate
Castleman's disease or angiofollicular lymph node hyperplasia is a rare clinical entity that may present in many sites and with a variety of symptoms. We report here a case of unicentric Castleman's disease (hyaline vascular pattern) located in the mediastinum as a solitary mass. The patient was a Caucasian female 58 years old presented after incidental discovery of the mass in a x-ray. In Castleman's disease surgery is generally recommended for localised lesions to remove the mass as completely as possible reserving other treatment modalities for unresectable cases or multicentric disease.
Castleman's Disease (CD) is a rare lymphoproliferative disorder accompanied by marked systemic inflammatory response. Morphological diagnosis of CD requires biopsy of the whole of the involved lymph node tissue. Three histologic variants have already been described in CD morphology (hyaline vascular, plasma-cell, and mixed). In this study, we report a case of a multicentric Castleman's disease of the plasma cell variant type with negative Herpes Virus 8. The clinical presentation of this patient was of systemic amyloidosis as a result of both a delayed diagnosis and medical management. Previously described cases of CD with secondary amyloidosis have been of the localized type. Regardless, long-standing clinical remission of CD by cytotoxic drugs and anti-CD20 antibody therapy was achieved, but the nephrotic syndrome remained irreversible.