Report on slide session, British Society for Haematology, 43rd Annual Scientific Meeting, Glasgow, 2003

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Report on slide session, British Society for Haematology, 43rd Annual Scientific Meeting, Glasgow, 2003
  Report on Slide Session, British Society forHaematology, 42nd Annual Scientific Meeting,Brighton, 2002 B. J. BAIN,D. WINFIELD,J. MURRAY,A. EDEN,E. PSIACHOU-LEONARD,M. GILLEECE,V. DEVALIA,C. HATTON,H. EAGLETON,S. RASSAN Each year, at the Annual Scientific Meeting of the BritishSociety for Haematology, there is an educational session inwhich two experts discuss the morphological features of blood films or bone marrow biopsy sections from patientswho have presented a diagnostic problem or who areotherwise instructive. The experts are given no informa-tion beyond the brief details provided to all participantswho review the slides before the meeting. After thediscussants have given their opinions, the case contributorpresents further details and gives the final diagnosis. Thisreport follows the format of the meeting so that the readercan reach a provisional diagnosis for him or herself beforethe definitive diagnosis is revealed. Case 1 The patient was a 57-year-old Caucasian male whopresented with a 3-week history of lethargy, night sweatsand weight loss. He was found to have massive hepato-splenomegaly. FBC was: WBC 195  ·  10 9 /l, Hb 15 g/dland platelet count 63  ·  10 9 /l. B-cell markers were neg-ative (case contributed by Dr A. Eden, Southend).The discussant (JM) illustrated medium-sized lymphoidcells with plentiful cytoplasm, azurophilic granules,cytoplasmic vacuolation and slightly irregular nucleiwith nucleoli (Figure 1). He favoured an aggressiveT-cell leukaemia (Gentile  et al. , 1994). He felt confidentin his diagnosis, as he recognized this case as a patientwho had recently been published in the British Journalof Haematology (Gupta, Mills & Eden, 2001). Thesecond discussant (DW) was left with little choice butto concur. Case 2 The patient was a 6-year-old Greek girl who presentedwith a 6-day history of fever. She was found to have mildcervical lymphadenopathy and the spleen was palpable3 cm below the left costal margin. FBC was: WBC290  ·  10 9 /l, Hb 7.3 g/dl, MCV 77 fl and platelet count307  ·  10 9 /l (case contributed by Dr Elene Psiachou-Leonard, Southampton).The discussant (DW) illustrated marked leucocytosiswith neutrophilia, eosinophilia, basophilia and numerousgranulocyte precursors (Figure 2). Monocytes were notprominent and dysplasia was minor. He thought thatthere were no features to suggest that this was reactive,the presence of immature granulocytes was not compat-ible with a diagnosis of chronic neutrophilic leukaemiaand the age and lack of monocytosis did not favour adiagnosis of juvenile myelomonocytic leukaemia or themonosomy 7 syndrome. He thought that, despite thechild’s age, the findings were totally typical of Philadel-phia-positive chronic myeloid leukaemia. The seconddiscussant (JM) was in complete agreement. A memberof the audience commented that this type of leukaemiawas in fact the most common myeloproliferative disorder Accepted for publication 5 February 2003Correspondence: Dr B.J. Bain, Department of haematology, St Mary’sHospital, Praed Street, London, W2 1NY, UK. E-mail: Clin. Lab. Haem. 2003,  25,  221–226   2003 Blackwell Publishing Ltd. 221  in children and the discussants should not hesitate tomake this diagnosis. Case 3 The patient was a 70-year-old man with known meta-static carcinoma of the prostate. A routine FBC hadshown: WBC 12.9  ·  10 9 /l, Hb 11.8 g/dl and plateletcount 169  ·  10 9 /l. He had moderate splenomegaly but nohepatomegaly or lymphadenopathy (case contributed byDr Maria Gilleece, Bangor).The discussant (JM) illustrated lymphoid cells withmoderately abundant, weakly basophilic, hairy cytoplasm(Figure 3). The majority of these cells had fairly prominentnucleoli and some appeared to have azurophilic granules.Two per cent of peripheral blood cells were monocytes(monocyte count 0.25  ·  10 9 /l). He considered the differ-ential diagnosis to be hairy cell leukaemia, hairy cellvariant or splenic lymphoma with villous lymphocytes(SLVL). He favoured hairy cell variant on the grounds of the easily detectable nucleoli and the fairly high count of neoplastic cells. DW was inclined to the view that this wasSLVL. A majority of the audience, having heard thediscussion, favoured a diagnosis of hairy cell variant but asignificant minority favoured each of the other twodiagnoses. Case 4 The patient was a 51-year-old woman with a past historyof polycythaemia vera who had been treated withhydroxyurea and had subsequently required splenectomyfor progressive splenomegaly. Later in the course of theillness, she had developed a haemorrhagic tendency. Theautomated platelet count was 15–30  ·  10 9 /l but amanual platelet count was 80  ·  10 9 /l (case contributedby Dr V Devalia, Bridgend).The discussant (DW) illustrated giant, poorly granula-ted platelets and fragments of megakaryocyte cytoplasm.He thought there were some megakaryoblasts showingcytoplasmic blebs (Figure 4). There was also dysgranulo-poiesis with hypogranular and Pelger-Hue¨t forms, hyper-segmented neutrophils and macropolycytes. Features of hyposplenism were apparent but teardrop poikilocytes Figure 1.  Peripheral blood film from case 1 showing medium-sized, pleomorphic, nucleolated lymphoid cells, some withazurophilic granules. Figure 2.  Peripheral blood film from case 2 showing a spectrumof cells from various granulocytic lineages. BSH Slide Session, 2002 222   2003 Blackwell Publishing Ltd.,  Clin. Lab. Haem. ,  25 , 221–226  were not prominent. He suspected transformation tomyelofibrosis or myelodysplasia with a major megakaryo-cyte abnormality and wondered whether the hyperseg-mented neutrophils might be the result of hydroxyureatherapy. JM generally concurred but was less impressed bythe dysgranulopoiesis. Case 5 The patient was a 17-year-old boy who presented with alarge mediastinal mass and a pleural effusion. FBC was:WBC 6.8  ·  10 9 /l, Hb 13.8 g/dl and platelet count232  ·  10 9 /l (case contributed by Dr C. Hatton and DrHelen Eagleton, Oxford).The discussant (JM) noted the presence of abnormalmedium-sized lymphoid cells with quite marked cytoplas-mic basophilia and prominent vacuolation (Figure 5).Some nuclei were slightly indented and rare cells appearedto have a convoluted nucleus. He thought that thedifferential diagnosis was precursor T-lymphoblastic leuk-aemia/lymphoma, precursor B-lymphoblastic leukaemia/ Figure 4.  Peripheral blood film from case 4 showing large andagranular platelets and a large immature cell with cytologicalfeatures suggesting that it is a megakaryoblast. Figure 3.  Peripheral blood film from case 3 showing (a) twonucleolated lymphoid cells with plentiful   hairy   cytoplasm; (b)three lymphoid cells with less cytoplasm but with one of the threehaving   villous   cytoplasm and a nucleolus. B. J. Bain  et al.  223   2003 Blackwell Publishing Ltd.,  Clin. Lab. Haem. ,  25 , 221–226  lymphoma and Burkitt’s lymphoma. He considered thepossibility of Hodgkin’s disease or sclerosing mediastinalB-cell lymphoma but the presence of abnormal cells in thecirculation made these diagnoses improbable. The clinicalfeatures clearly pointed to a precursor T-lymphoblasticleukaemia/lymphoma but the morphology was rathersuggestive of Burkitt’s lymphoma. Overall, given that thiswas a BSH case and nothing is ever straightforward, hethought this would probably turn out to be B-lineagedisease DW concurred. He also suspected that a diagnostictrap had been laid and overall he thought this was   more Bthan T  . Case 6 The patient was a 28-year-old Turkish Cypriot man withthalassaemia intermedia who had suffered steadily wor-sening anaemia and had required splenectomy. He alsosuffered from osteoporosis. Hb was 6.2 g/dl and MCV84 fl. The total nucleated cell count was high, as a resultof numerous circulating nucleated RBCs (case contributedby S. Rassam, Sidcup).The discussant (DW) noted that the trephine biopsysections showed erythroid hyperplasia with clusters of immature erythroid cells; he wondered if these weremegaloblasts and if the patient had neglected to take hisfolic acid. In addition, there were considerable numbersof large macrophages with voluminous pale cytoplasmand eccentric nuclei; some of these appeared foamy(Figure 6). These cells had the appearance of storage cellsand he thought the differential diagnosis was between astorage disease (Gaucher’s disease or Niemann-Pickdisease) and pseudo-Gaucher cells as a result of increasedcell turnover. Such cells have been reported in thalas-saemia major. He wondered if the bone disease mighthave been osteolytic lesions as a result of Gaucher’sdisease rather than osteoporosis. The clinical features didnot appear compatible with type A or type B Niemann-Pick disease but the possibility of Gaucher’s disease ortype C Niemann-Pick disease was not excluded. JM alsofavoured a storage disease, perhaps Gaucher’s disease,rather than the abnormal macrophages representingpseudo-Gaucher cells. Figure 6.  Trephine biopsy section from case 6 showing sheets of altered macrophages with voluminous pale pink cytoplasm. Figure 5.  Peripheral blood film from case 5 showing two largeimmature lymphoid cells with basophilic cytoplasm and cyto-plasmic vacuolation. BSH Slide Session, 2002 224   2003 Blackwell Publishing Ltd.,  Clin. Lab. Haem. ,  25 , 221–226  Discussion and final diagnoses Case 1 The chairman (BB) reported, on behalf of the casecontributor (AE), that this was indeed the published case,and commented that the virtues of reading the British Journal of Haematology had been demonstrated. Theneoplastic cells expressed CD2, CD3, CD7, CD8, T-cellreceptor  ab , CD11b, CD16, CD56 and CD57.Most patients with T-lineage large granular lymphocyteleukaemia have a disease that runs a relatively chroniccourse but NK-like T-cell lymphoma is much moreaggressive. The patient did not respond to four coursesof combination chemotherapy (CHOP regime – cyclo-phosphamide, doxorubicin, vincristine and prednisolone)and died within 5 months of presentation. Case 2 BB reported, on behalf of the case contributor (EP-L),that the child had indeed suffered from Philadelphia-positive chronic myeloid leukaemia. The  BCR-ABL  rear-rangement had also been demonstrated. BB mentionedthat, at a previous session of the conference, Prof. IreneRoberts had reported that 45 children with chronicgranulocytic leukaemia had been seen at HammersmithHospital, London, in the last 20 years. The youngestreported patient with this disease was only 3 monthsold. Case 3 BB reported, on behalf of the case contributor (MG), thatthe neoplastic cells expressed B-cell markers (CD19,CD20, CD22 and CD23); they expressed CD10 weaklybut not FMC7; they expressed two hairy cell markers(CD11c and CD103) but not CD25 and were positive fortartrate-resistant acid phosphatase. The chairman sup-posed that, if one accepted that consensus represented  truth  , the diagnosis must be hairy cell variant. Prof. D.Catovsky was unable to be present at the meeting butexamined the slides. He thought that this was aborderline case, so was difficult to classify with certaintyas hairy cell leukaemia or hairy cell variant; theprominent nucleolus (uncommon in hairy cell leukae-mia), the lack of expression of CD25 and the lack of monocytopenia might tilt the balance to hairy cellleukaemia variant. The patient declined treatment butremained relatively well with stable peripheral countsand splenomegaly. Case 4 The case contributor (DV) reported that the patient haddeveloped post-polycythaemia myelofibrosis. She had notbeen on hydroxyurea at the time the blood film was made.Despite the platelet count of 80  ·  10 9 /l and the numerouslarge platelets, she had developed a marked bleedingtendency. He attributed this to the largely agranularplatelets being functionally defective. Cytogenetic analysishad been normal early in the course of the disease but 20q-had been detected later. The patient remained reasonablywell, having benefited from thalidomide therapy. Case 5  HE reported that the patient also had lymphadenopathy.Cytologicallysimilarcellswereseenintheperipheralblood,bonemarrowaspirateandpleuralfluid.Theneoplasticcellsexpressed CD2, CD3, CD5, CD7, CD10 and terminaldeoxynucleotidyl transferase (Figure 7). CD19 and CD20were not expressed. The final diagnosis was thereforeprecursor T-lymphoblastic leukaemia/lymphoma.    1   0    4    1   0    3    1   0    2    C   D   5   P   E   C   D   1   1   7   P   E CD15 FITCCD19 FITC    1   0    1    1   0    0    1   0    4    1   0    3    1   0    2    C   D   7   P   E   C   D   1   0   P   E   C  y   t   C   D   3   P   E   1   0    1    1   0    0    1   0    4    1   0    3    1   0    2    C   D   3   4   P   E   1   0    1    1   0    0    1   0    4    1   0    3    1   0    2    1   0    1    1   0    0    1   0    4    1   0    3    1   0    2    1   0    1    1   0    0 10 4 10 3 10 2 10 1 10 0 10 4 10 3 10 2 10 1 10 0 CD10 FITCCD2 FITC10 4 10 3 10 2 10 1 10 0 10 4 10 3 10 2 10 1 10 0 CytTdT FITCCD20 FITC10 4 10 3 10 2 10 1 10 0 10 4 10 3 10 2 10 1 10 0    1   0    4    1   0    3    1   0    2    1   0    1    1   0    0 Figure 7.  Immunophenotyping of neoplastic cells from case 5. B. J. Bain  et al.  225   2003 Blackwell Publishing Ltd.,  Clin. Lab. Haem. ,  25 , 221–226
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