Lege Artis Medicinae

[Diagnostic challenges in our adolescent patient with lymphoma]

MAGYARI Ferenc, RAJNAI Hajnalka, BARNA Sándor, MILTÉNYI Zsófia, VÁRÓCZY László, CSOMOR Judit, UDVARDY Miklós, ILLÉS Árpád

APRIL 20, 2014

Lege Artis Medicinae - 2014;24(04)

[OBJECTIVES - Unclassifiable B-cell lymphoma, which shows intermediate features typical for both diffuse, large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (HL) is a novel category of diffuse, large B-cell lymphomas (DLBCL/HL), described in the WHO classification in 2008. This rare type of lymphomas presents peculiar clinical, morphological and immunophenotypical patterns, previously called gray-zone lymphomas. CASE REPORT - In December 2011 a 17- year old boy was diagnosed with mixedcellularity subtype of classical HL on the basis of left inguinal lymph node biopsy. Staging examinations revealed a IV/BXS (abdominal bulky) stage disease with unfavourable prognosis. Because of the unusally extended disease (nodal-extranodalbulky), a histological revision was performed. After a half course of ABVD chemotherapy the patient’s symptoms disappeared and the sizes of the involved lymph nodes decreased. On the basis of the histological revision, the diagnosis was changed to DLBCL/HL, so the treatment was modified to R-CHOP-14 regimen. After 3 cycles of R-CHOP-14 a complete metabolic remission (CMR) was achieved, which was confirmed by a 18FDG-PET/CT scan. Staging examinations after further 4 cycles of RCHOP- 14 therapy showed that the patient was still in CMR, but a PET-negative large mass (7×3 centimeter) still remained visible in the abdominal region. Considering this residual tumour and the agressive subtype of lymphoma the patient was referred for an autologous haemopoietic stem cell transplantation (AHSCT). After 2 cycles of R-DHAP regimen, successful CD34- positive stem cell collection was performed in August 2012. In September 2012, a RLAM-BEAM conditioning was performed followed by AHSCT. Posttransplantation 18FDG-PET/CT scan revealed further morphological regression, no symptom of the underlying disease appeared and the patient is in complete remission for 15 months. CONCLUSIONS - This case exemplifies that in case of atypical clinical findings and unusual progress of the disease it might be worthwile to re-evaluate the case and the (histological) diagnosis, which requires a close cooperation between the clinician and the pathologist.]

COMMENTS

0 comments

Further articles in this publication

Lege Artis Medicinae

[Antibody deficiencies, investigation, therapy]

MOSDÓSI Bernadett, NYUL Zoltán

[Inborn antibody deficiencies represent the most common primary immunodeficiency diseases. This heterogeneous group of disorders is characterised by insufficient antibody production resulting from a genetic defect of B cell differentiation or from abnormal interactions between B and T cells. The clinical manifestations are recurrent, often severe upper and lower respiratory tract infections, but chronic diarrhea and autoimmune diseases might also occur. Timely diagnosis and therapy significantly decrease morbidity, thus increasing life expectancy and improve quality of life.]

Lege Artis Medicinae

[Vaccine coverage of invasive and carried Streptococcus pneumoniae isolates in Hungary]

TÓTHPÁL Adrienn, LAUB Krisztina, KARDOS Szilvia, NAGY Károly, DOBAY Orsolya

[Vaccination against Streptococcus pneumoniae will be mandatory for infants and children from 1 July 2014 in Hungary. Since the introduction of the conjugate vaccine against Pneumococcus, the distribution of Pneumococcus serotypes has changed significantly. Surveillance data from the National Centre for Epidemiology show that certain vaccine-type pneumococcal serotypes have almost disappeared owing to the immunization program, while other serotypes have emerged. Nowadays serotype 3 and the multiresistant 19A serotype are the most frequent causes oif invasive infections. A study analysing the nasal carriage of children attending kindergartens also confirmed the decline of vaccine- serotypes parallel to vaccination coverage and the simultaneous emergence of previously very types, such as 19A. The most recent study examining nasal carriage of children attending nursery (age < 3 years) showed that the 13-valent conjugate vaccine has efficiently decreased the carriage of serotypes 3 and 19A, verifying that the immunisation program should be continued using the vaccine providing the broadest coverage against Pneumococcus.]

Lege Artis Medicinae

[The World of Toulouse-Lautrec – Lithographs in the Museum of Fine Arts ]

NAGY Zsuzsanna

Lege Artis Medicinae

[Viral hepatitis in focus: messages of the hepatological conference about screening, diagnostics and possibilities of effective therapy]

NEMESÁNSZKY Elemér

Lege Artis Medicinae

[The Cultural History of the Discovery of Insulin ]

FELKAI Péter

All articles in the issue

Related contents

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

[The role of sleep in the relational memory processes ]

CSÁBI Eszter, ZÁMBÓ Ágnes, PROKECZ Lídia

[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

Simultaneous subdural, subarachnoideal and intracerebral haemorrhage after rupture of a peripheral middle cerebral artery aneurysm

BÉRES-MOLNÁR Anna Katalin, FOLYOVICH András, SZLOBODA Péter, SZENDREY-KISS Zsolt, BERECZKI Dániel, BAKOS Mária, VÁRALLYAY György, SZABÓ Huba, NYÁRI István

The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.