27741115 Visualization

A 54 Age - year Age - old Age patient was admitted Clinical_event for fever Sign_symptom , diarrhea Sign_symptom , and acute Detailed_description renal Disease_disorder failure Disease_disorder .
He had no History significant History medical History history History , did not take any medication Medication , and had History not History traveled History recently History .
He had no Family_history relevant Family_history familial Family_history history Family_history .
Fifteen Date days Date before Date his admission Clinical_event , he developed fever Sign_symptom , fluctuant Detailed_description rash Sign_symptom , diarrhea Sign_symptom , and pain Sign_symptom in the joints Biological_structure .
On admission Clinical_event , blood Diagnostic_procedure pressure Diagnostic_procedure was 140/70 Lab_value mm Lab_value Hg Lab_value , temperature Diagnostic_procedure 39°C Lab_value .
Physical Diagnostic_procedure examination Diagnostic_procedure showed severe Severity edema Sign_symptom involving both lower Biological_structure and Biological_structure upper Biological_structure limbs Biological_structure , as well as ascites Sign_symptom and pleural Sign_symptom effusion Sign_symptom .
Cervical Biological_structure and axillar Biological_structure infracentimetric Qualitative_concept lymphadenopathies Disease_disorder were present, together with hepatosplenomegaly Disease_disorder , confirmed by computed Diagnostic_procedure tomography Diagnostic_procedure ( CT Diagnostic_procedure ) scan.
Bilateral Detailed_description arthritis Disease_disorder of the ankles Biological_structure resolved spontaneously.
Blood Diagnostic_procedure analysis Diagnostic_procedure revealed (Table ​(Table1):1): leukocytosis Sign_symptom , normal Lab_value hemoglobin Diagnostic_procedure and platelet Diagnostic_procedure levels with no biologic sign of hemolysis Sign_symptom , elevated Lab_value C Diagnostic_procedure - reactive Diagnostic_procedure protein Diagnostic_procedure , low Lab_value albumin Diagnostic_procedure , elevated Lab_value serum Detailed_description creatinin Diagnostic_procedure , mild Severity proteinuria Sign_symptom and no hematuria Sign_symptom .
Despite intravenous Detailed_description rehydration Therapeutic_procedure using saline Medication and albumin Medication , renal Diagnostic_procedure function Diagnostic_procedure worsened Lab_value and the patient required dialysis Therapeutic_procedure .
While diagnostic Diagnostic_procedure investigations Diagnostic_procedure were performed, hemiplegia Disease_disorder occurred: brain Biological_structure MRI Diagnostic_procedure showed multifocal Detailed_description ischemic Detailed_description lesions Sign_symptom .
Echographic Detailed_description and rythmologic Detailed_description studies Diagnostic_procedure ruled out any cardiologic Disease_disorder cause Disease_disorder for the stroke Disease_disorder .
A renal Biological_structure biopsy Diagnostic_procedure was performed (Fig.1A and B).
Periodic Diagnostic_procedure acid Diagnostic_procedure – Schiff Diagnostic_procedure ( PAS Diagnostic_procedure ) staining showed endotheliosis Disease_disorder in all glomeruli Biological_structure (Fig.1A, arrows), associated with mesangiolysis Disease_disorder and double Sign_symptom contours Sign_symptom on silver Diagnostic_procedure staining Diagnostic_procedure (Fig.1B, arrows), and no arteriolar Biological_structure thrombus Sign_symptom .
A moderate Severity CD20+ Detailed_description B Sign_symptom lymphocyte Sign_symptom infiltrate Sign_symptom was present in the interstitium Biological_structure , with a peritubularcapillaritis Sign_symptom .
Immunofluorescence Diagnostic_procedure study Diagnostic_procedure did not show any deposit Sign_symptom .
Lymph Biological_structure node Biological_structure biopsy Diagnostic_procedure (Fig.2A and B) showed abnormal Sign_symptom follicles Biological_structure with hyalinization Sign_symptom of germinal Biological_structure center Biological_structure , and an onion Sign_symptom - skin Sign_symptom aspect Sign_symptom of the mantle Biological_structure zone Biological_structure .
Clinical presentation and lymph Biological_structure node Biological_structure histology Diagnostic_procedure were consistent with hyaline Detailed_description - vascular Detailed_description multicentric Disease_disorder Castleman Disease_disorder disease Disease_disorder ( MCD Disease_disorder ).
HIV Detailed_description and HHV-8 serology Diagnostic_procedure , as well as HHV-8 lymph Biological_structure node Biological_structure tissue Diagnostic_procedure staining Diagnostic_procedure , were negative Lab_value .
Serum Detailed_description vascular Diagnostic_procedure endothelium Diagnostic_procedure growth Diagnostic_procedure factor Diagnostic_procedure ( VEGF Diagnostic_procedure ) was highly Lab_value elevated Lab_value .
There was no biologic manifestation of thrombotic Disease_disorder microangiopathy Disease_disorder ( TMA Disease_disorder ), but the renal Biological_structure biopsy Diagnostic_procedure and brain Biological_structure MRI Diagnostic_procedure were consistent with glomerular Biological_structure and neurologic Biological_structure TMA Disease_disorder lesions.
No other apparent cause of thrombotic Detailed_description microvascular Sign_symptom involvement Sign_symptom was noted.
Notably, there was no evidence for infection with shiga Disease_disorder toxin Disease_disorder - producing Disease_disorder germs Disease_disorder , ADAMTS-13 activity Diagnostic_procedure was decreased Lab_value up Lab_value to Lab_value 14% Lab_value but remained above Lab_value 5% Lab_value , and the alternative Diagnostic_procedure complement Diagnostic_procedure pathway Diagnostic_procedure was normal Lab_value .
Plasma Therapeutic_procedure exchange Therapeutic_procedure was initiated due to the kidney Biological_structure histological lesions Sign_symptom and the multifocal Detailed_description ischemic Detailed_description brain Biological_structure lesions Sign_symptom .
It was discontinued Detailed_description after Date 1 Date month Date after the onset of the disease, since the patient's condition Diagnostic_procedure remained stable Lab_value .
Furthermore, chemotherapy Medication including 6 courses of rituximab Medication ( 375 Dosage   mg/m2 Dosage ), cyclophosphamide Medication ( 750 Dosage   mg/m2 Dosage ), and dexamethasone Medication ( 40 Dosage   mg/day Dosage from day Duration 1 Duration to Duration day Duration 4 Duration ) was started together with plasma Therapeutic_procedure exchanges Therapeutic_procedure (chemotherapy was performed immediately after plasma exchanges).
Courses Coreference were performed every Frequency 3 Frequency weeks Frequency .
Clinical manifestations of vascular Disease_disorder leak Disease_disorder syndrome Disease_disorder regressed, renal Diagnostic_procedure function Diagnostic_procedure normalized Lab_value , and serum Detailed_description VEGF Diagnostic_procedure level decreased Lab_value to 825 Lab_value   pg/mL Lab_value after 1 Quantitative_concept course Quantitative_concept of chemotherapy Medication .
The patient was discharged Clinical_event 43 Date days Date after Date admission.
No neurological Disease_disorder event Disease_disorder occurred after initiating the treatment.
Thoraco Biological_structure - abdominal Biological_structure CT Diagnostic_procedure scan was performed after 6 Quantitative_concept courses Quantitative_concept of chemotherapy Medication , showing a normal Lab_value liver Biological_structure and spleen Biological_structure size Diagnostic_procedure and no lymph Biological_structure node Biological_structure enlargement Sign_symptom .
One Date year Date after Date the diagnosis Clinical_event , remission of MCD Disease_disorder is persistent and plasma Detailed_description creatinine Diagnostic_procedure is 86 Lab_value   μmol/L Lab_value , with no proteinuria Sign_symptom .