EAHP 2020 workshop proceedings, pediatric myeloid neoplasms.

The first section of the bone marrow workshop of the European Association of Haematopathology (EAHP) 2020 Virtual Meeting was dedicated to pediatric myeloid neoplasms. The section covered the whole spectrum of myeloid neoplasms, including myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPN), myelodysplastic/myeloproliferative neoplasms (MDS/MPN), and acute myeloid leukemia (AML). The workshop cases are hereby presented, preceded by an introduction on these overall rare diseases in this age group. Very rare entities such as primary myelofibrosis, pediatric MDS with fibrosis, and MDS/MPN with JMML-like features and t(4;17)(q12;q21); FIP1L1::RARA fusion, are described in more detail.

[Hodgkin and the classification of malignant lymphomas: how the British physician also became associated with later discovered lymphomas]. / Hodgkin en de classificatie van maligne lymfomen.

Thomas Hodgkin was the first to describe a malignant lymphoma, which would later carry his name. Over the course of time, other lymphoid malignancies were recognised that showed no similarity with Hodgkin's disease. They were subsequently named after the - at that time - applicable morphological nomenclature of the associated cells. Later, nomenclature also took immunological features into consideration. However, we still describe the group of lymphomas recognised after Hodgkin's discovery as 'not being Hodgkin's disease', i.e. non-Hodgkin lymphoma. We feel it is unjust that not many people know about the man behind this prominent disease. In this article, an historic overview is given of Thomas Hodgkin, 'his' lymphoma and the other malignant lymphomas.

Pulmonary interstitial glycogenosis in identical twins.

We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally. Radiological examination showed a reticular nodular interstitial pattern on chest radiography. High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants. Lung biopsies showed a similar histology. There was diffuse interstitial thickening of the alveolar septa by mesenchymal cells, without prominent hyperplasia of type 2 pneumocytes, and without airspace exudates. Sections were periodic acid-Schiff (PAS)-positive within the cytoplasm of interstitial cells, indicating the presence of glycogen. Thus the diagnosis of pulmonary interstitial glycogenosis was made. Both infants were treated with glucocorticoids and had a favorable outcome. We speculate that pulmonary interstitial glycogenosis could be a histopathological form of chronic lung disease (CLD) of infancy.

[Tropical or non-tropical sprue?]. / Inheemse of uitheemse spruw?

A 57-year-old Dutch man presented with weight loss and fatigue 6 months after a visit to West Papua, when he had suffered from serious diarrhoea. Macrocytic anaemia and vitamin B12 deficiency were diagnosed. A gastroduodenoscopy with biopsies of the small intestine was performed revealing no macroscopic abnormalities but partial villous atrophy was found microscopically, suggesting tropical sprue or coeliac disease. Antibodies against endomysium and tissue transglutaminase were negative, ruling out coeliac disease. The patient was successfully treated with vitamin B12, folic acid and doxycycline. This case shows that tropical sprue should be considered in the differential diagnosis of chronic diarrhoea in patients with a history of travel in tropical regions. The most frequent medical problem that travelers to the tropics experience is diarrhoea with an incidence of 30%. A small proportion of these patients eventually present with chronic diarrhoea. At that moment, the relation to their previous travelling may not be immediately clear. One of the causes of this chronic diarrhoea to be considered is tropical sprue.

Cyclooxygenase-2 mediated regulation of E-cadherin occurs in conventional but not early-onset gastric cancer cell lines.

BACKGROUND: COX-2 and E-cadherin, involved in invasion and metastasis, are molecules critical for gastric carcinogenesis. A relationship between them is documented in non-small cell lung and prostate cancer. We present novel evidence of a relationship between COX-2 and E-cadherin expression in gastric cancer. METHODS: Using qPCR and Western blots analysis on celecoxib and PGE2 treated and untreated gastric cancer cell lines derived from tumours of the intestinal type (MKN45, MKN28, AGS3, MKN7) and immunohistochemistry of 178 gastric cancers on tissue microarrays (TMA), we examined the COX-2/E-cadherin relationship. RESULTS: Down-regulation of COX-2 by celecoxib led to up-regulation of E-cadherin mRNA and protein levels in conventional gastric cancer cell lines, whereas expression was down regulated in the early-onset gastric cancer (EOGC) cell line. Immunohistochemistry on TMAs of 178 gastric cancers showed no correlation between COX-2 and E-cadherin expression in the conventional or early gastric cancer groups. CONCLUSION: The results suggest that COX-2 has an impact on transcriptional regulation of E-cadherin in gastric cancer and our findings further highlight the intriguing nature of EOGCs which appear to have a molecular phenotype distinct from conventional gastric cancer. In addition, our findings also suggest that reduction of COX-2 using nonsteroidal anti-inflammatory drugs in gastric cancer chemoprevention may only be relevant for older patients.

Giant rectal gastrointestinal stromal tumors: a report of two cases.

Giant gastrointestinal stromal tumors (GISTs) of the rectum are rare and often difficult to remove surgically. At the time metastases are found, GISTs are considered to be incurable and until recently no adequate therapy was of any value for these patients. Recently, imatinib was introduced: a signal transducing inhibitor acting specifically on the KIT-tyrosine kinase, which can be used to downsize giant GIST (neo-adjuvant) before surgery or induce stable disease in case of metastases with few minor side-effects. Two patients with giant rectal GIST are presented, one of which was treated before the imatinib era, the other when imatinib was available.