Illustrated Pathology of the Spleen Bridget S

Cambridge University Press 0521622271 - Illustrated Pathology of the Spleen Bridget S. Wilkins and Dennis H. Wright Index More information

Index

Note: page numbers in italics refer to ﬁgures and tables.

abscess cystic change 153 formation in spontaneous rupture 43 metastasis; differential diagnosis 152 mycotic 152 subdiaphragmatic 159 tuberculous 152 acanthocytes 24 acquired immune deficiency syndrome (AIDS) 66–70 bacillary angiomatosis 69–70 CD8-positive T cells 67–8 Kaposi’s sarcoma 141 leishmaniasis 73, 175 opportunistic infection 68–70 tumours 70 white pulp atrophy 68 see also human immunodeficiency virus (HIV) infection actin see alpha-smooth muscle actin (␣-SMA) acute leukaemia 122 acute lymphoblastic leukaemia 122 immunohistochemistry 122, 123 acute megakaryoblastic leukaemia (AML), AML-M7 subtype 121 acute myeloid leukaemia 122, 169 immunohistochemistry 122, 123 alpha-smooth muscle actin (␣-SMA) 16, 17, 21 PALS dendritic network 30 spindle cells 30, 32 amyloid staining 144 types A and L 155 amyloid tumour 154 amyloidosis 154–5 anatomy of spleen 4, 13–17 angioma cystic change 153 littoral cell 140–1

181

Index 182

angiomatosis migration 22–3 bacillary 69–70 red pulp 22 diffuse 139–40 white pulp 22–3 angiosarcoma 140–1, 141–3 B-prolymphocytic leukaemia 82 Thorotrast-induced 159 bacillary angiomatosis 69–70 ankyrin 52 bacteria, encapsulated 11 anticoagulant therapy, spontaneous rupture 43 bacterial endocarditis 147 antigen presentation site 16 splenic atrophy 158 antigen retrieval methods 9 band 3 52 antigen-presenting cells (APC) 22 barrel hoop fibres 16 apoptosis 23 Bartonella henselae 69–70 atrophy of spleen 23, 157–9 bcl-2 86, 87 due to Thorotrast exposure 158–9 bilateral spleen 155 in HIV infection 68 biochemical analysis 9 in sickle cell anaemia 50 biopsy 10–11 auto-antibodies Birbeck granules 137, 138 blood cell coating 52 blast cells 40, 42 in non-Hodgkin’s lymphoma 53 in acute leukaemia 122 auto-immune cytopenia 26 in Epstein–Barr virus infection 71 auto-immune haemolytic anaemia (AIHA) 2, 6, 47, 51–2 in lymphoplasmacytoid lymphoma 92 atrophy of spleen 157 in marginal zone lymphoma 90 causes 52–3 in mast cell leukaemia 120 cordal macrophages 54, 57 blood cells erythrocytosis 25 reservoir 5 erythrophagocytosis 54, 56 see also red cells; sequestration incidental EMH 178 blood lakes, hairy cell leukaemia 94–5, 97, 98, 173 intrasinusoidal red cell phagocytosis 54–5, 56 blood supply see vascular supply of spleen macroscopic appearance of spleen 53 bone marrow hyperplasia 25 microscopic features 56, 60 Bruton’s X-linked agammaglobulinaemia 64, 65 white pulp changes 55–6 Burkitt’s lymphoma 77 autolysis, splenic 160–1 post-mortem 73–4 cancers metastasis sources 152 B cell lymphocytic lymphoma (B-CLL) 77, 78, 79–80, routes of spread 151 81–2 capsular damage 6 chromosome deletions 81, 82 capsule 14 distinction from mantle cell lymphoma 83 cassettes, for fixation 8 genetic features 81–2 cat spleen 5 IgG gene mutations 81–2 cat-scratch disease 69 immunophenotype 81 CD4-positive T cells 15, 16 para-immunoblasts 78, 80, 81 AIDS 68 trabecular infiltration 167 CD8 expression 15, 16 trisomy 12 81, 82 CD8-positive T cells, AIDS 67–8 B cell lymphoma CD68 expression 20 nodular lymphocyte and histiocyte predominant centroblasts 27 Hodgkin’s disease 105 in follicle centre cell lymphoma 86, 89 see also lymphoma; large B cell lymphoma; small B cell centrocytes 23, 27 lymphoma in follicle centre cell lymphoma 89 B cells 15 ceroid 131, 134, 135 in inherited immunodeficiency states 64–5 accumulation 127, 175, 176 marginal zones 28, 29 ceroid histiocytes 128

183 Index

ceroid histiocytosis 55, 126, 130, 131, 134, 135 differential diagnosis 163 chemotherapy, cytotoxic 66 analytical approach 163 chronic granulocytic leukaemia (CGL) 114 cordal macrophage disorders 173, 175–6 chronic lymphocytic leukaemia (CLL) 164, 168 EMH 176–7, 178 hilar lymph nodes 172 germinal centres 164, 165, 166 mantle zones 166, 167 granulomas 173, 175–6 circulatory pathways hilar lymph nodes in splenic lymphoma closed/open 19, 20 171–2 fast transit 26 macrophages 175–6 red pulp 19, 20 mantle zones 166 closed circulation see circulatory pathways marginal zones 167 coeliac disease 158 peri-arteriolar lymphoid sheaths (PALS) 168 congenital absence of spleen 24 red pulp 169 congenital anomalies 155–6 diffluent spleen 74 connective tissue trabeculae 40 DiGeorge’s syndrome 64 cordal macrophages 20, 21 dog spleen 5 differential diagnosis of disorders 173, 175–6 Down’s syndrome see trisomy 21 in AIHA 54, 57 drug therapy, acquired immunodeficiency 65–6 in ceroid histiocytosis 135 Dutcher bodies 91, 92, 167 in HS 54, 55 dyserythropoiesis 47 in ITP 55, 58 in malaria 72 Echinococcus 154 in Niemann–Pick disease 130, 131 ectopia, splenic 156 pitting 24, 50 elderly patients, spleen size 39–40 platelet phagocytosis 55 embolism, spontaneous rupture 43 storage disorders 26, 126–8, 129, 130–1, 132–4, 135–6 endothelial cells cords of Billroth see splenic cords immunohistochemistry 31, 34 corticosteroids 6 in angiosarcoma 140, 141, 142, 143 in acquired immunodeficiency 65 in haemangioma 139, 140 in auto-immune disorders (AIHA+ITP) 56 in hereditary spherocytosis (HS) 54 in Felty’s syndrome 57 in littoral cell angioma 140, 141 in systemic lupus erythematosus 59 in lymphangioma 139, 140 tingible body macrophages 136 in Niemann–Pick disease 130, 132 white pulp microscopic changes 56 red pulp 41, 43 Crohn’s disease 158 in storage disorders 127 cryptococcosis 68–9 see also sinusoidal endothelium cyclin D1 85, 86 epithelial inclusion cysts 153–4 cystic lesions 154 epithelioid granulomas 125, 173 cysts 153–4 Hodgkin’s disease 173, 175 cytogenetic analysis 9 see also granulomas cytopenia Epstein–Barr virus (EBV) infection 44, 70–1 auto-immune 26 in autolysed tissue 161 peripheral blood 25 erythrocytes in incidental EMH 112 cytoplasmic inclusions erythrocytosis, secondary 25 ceroid histtocytoses 131 erythroid cells, dysplastic in significant extramedullary Gaucher’s disease 130 haemopoiesis (EMH) 115 Niemann–Pick disease 130, 132, 133 erythroid precursors 115, 160 erythrophagocytosis in AIHA 54, 56 dendritic cells, follicular 27 essential thrombocythaemia (ET) 114, 115, 117 description of spleen 7, 8 peliosis 118 development of spleen 17–19 Evans’ syndrome 47, 51, 53

Index 184

experimental animals 5 marginal zone differentiation 86, 168 haemopoiesis 18 mutations 87 extra-follicular focus, white pulp 22–3 red pulp 171 extramedullary haemopoiesis (EMH) 18, 111–15, 116, white pulp nodules 86, 88, 165 117 follicular dendritic cells 27 cytology 177 differential diagnosis 176–7, 178 Gamna–Gandy body 59, 145, 147, 148 distribution 177 foreign body giant cells 147, 148 incidental 18, 112–13, 176, 177, 178 perisplenitis 159, 160 causes 113–14 Gaucher’s cells 128, 129, 130, 175 megakaryocytes 111 Gaucher’s disease 126, 127–8, 129, 130 primitive myeloid cells 112–13 germinal centres 14, 15 significant 114–15, 116, 117, 178 atrophy in X-linked hyper-IgM syndrome 65 associated diseases 114 differential diagnosis 164, 165, 166 differential diagnosis 176, 177, 178 fibrinoid necrosis 74 subtypes 111 immunohistochemistry 27–8 extramedullary haemopoietic cells in autolysed tissue in post-traumatic spleen 36 160–1 involution 43 reactive changes 39 false cysts 153–4 glucosyl ceramide degradation enzyme deficiency 127 Felty’s syndrome 56–7, 97 granulocytes in EMH 177, 178 atypical 57 in incidental EMH 112–13 red pulp 61 granulomas 125–6 fibrinoid necrosis caseating 125–6 germinal centres 74 cystic change 153 malaria 72 differential diagnosis 173, 175–6 systemic lupus erythematosus 59 in Hodgkin’s disease 104, 105 fibrocongestive splenomegaly 145, 146 in selective IgA deficiency 64–5 fibrosis 157–9 red pulp 173, 175 in malaria 72 sarcoid-like 125–6 in systemic mastocytosis 119, 120 white pulp 173, 175 in Thorotrast-induced atrophy 159 see also epithelioid granulomas onion-skin 59 granulomatous inflammation 125–6 fibrous histiocytosis, malignant 141 granulopoietic cells in autolysis 160 fine needle aspiration cytology 10 fixation 3–4, 7–9 haemangioma 139–40, 152–3 optimization 7 haematoma partial 3, 4, 8 intrasplenic 38 primary 4 subcapsular 8 secondary 3 haemoglobin abnormalities 49–51 fixative sickle cell anaemia 49 penetration 3, 4 thalassaemias 50 follicle centre cell lymphoma (FCCL) 77, 78, 85–7, 88–9, haemolytic anaemia see auto-immune haemolytic 164, 165 anaemia (AIHA) bcl-2 86, 87 Haemophilus influenzae type B vaccination 6–7, 11, 48 centroblasts 86, 89 haemopoiesis, splenic 18 centrocytes 89 see also extramedullary haemopoiesis (EMH) differential diagnosis 86 haemopoietic cells genetic features 87 extramedullary 160–1 hilar lymph nodes 172 immunohistochemistry 123 immunophenotype 86–7 haemopoietic function in embryonic/fetal life 17–18

185 Index

haemopoietic lineages, antibodies reactive with 123 histoplasmosis 68–9 haemopoietic malignancy; implications of splenectomy Hodgkin’s disease 77, 78, 101, 104–5, 106 11–12 cystic nodules 153 haemopoietic stem cells 113–14, 115 genetic features 105 haemorrhage, parenchymal 147 granulomas 105, 173, 175 haemosiderin deposition immunophenotype 104–5 in AIHA 55 lymphocyte-rich classical 106 in cystic lesions 154 mutations 105 in HS 54 nodular lymphocyte and histiocyte predominant 105–6 in Gamna–Gandy bodies 147 nodular sclerosing 106 in Gaucher’s disease 128 staging laparotomy 101, 104, 105, 106 in malaria 72 variants 105–6 in peri-splenitis 159 Howell–Jolly bodies 24, 50 in sickle cell anaemia 50 human immunodeficiency virus (HIV) infection 66–70 haemozoin, malarial 72 ITP-like syndrome 53, 67–8 hairy cell leukaemia 28, 77, 78, 92, 94–5 peliosis 143 blood lakes 94–5, 97, 98, 173 progression to AIDS 68 cytology 95 splenic atrophy 68 differential diagnosis 171, 172–3 white pulp atrophy 68 genetic features 95 see also acquired immunodeficiency syndrome (AIDS) hairy cells 94, 95, 96, 97 hyaline change in white pulp modules 43 immunophenotype 95 hyalinosis, splenic vascular 144, 145, 154 marginal zone lymphoma differentiation 87 hydatid cysts 154 peliosis 173 hyper-IgM syndrome, X-linked 65 red pulp 94, 95, 98, 174 hypersplenism 2, 23, 25–6 reticulin pattern 174 causes 24 splenic cord 95, 98 diagnosis 25 tumour cells 94, 96 EMH 112 hamartoma 152–3, 154 Felty’s syndrome 56–7 Heinz bodies 24 primary 26 hepatosplenic ␥␦-T cell lymphoma 77, 100–1, 102, 169 secondary 26 genetic features 101 splenomegaly 47 immunophenotype 101 hypogammaglobulinaemia 126 tumour cells 103 hypoplasia of spleen 24 hereditary elliptocytosis (HE) 47, 51 hyposplenism 2, 23, 24–5 mutations 52 causes 24 hereditary spherocytosis (HS) 5, 11, 47, 51–2 clinical features 24 erythrocytosis 25 in amyloidosis 154 macroscopic appearance of spleen 53 in sickle cell anaemia 50 marginal zone expansion 56, 59 microscopic features 54, 55, 60 idiopathic thrombocytopenic purpura (ITP) 2, 6, 11, 47, mutations 52 51–2 red cell sequestration 54 causes 52–3 splenectomy 51 cordal macrophages 55, 58, 173 vaccine-related changes 48 in HIV infection 67–8 white pulp 55–6, 59 in secondary storage disorders 135 hilar lymph nodes 5, 8, 9 macroscopic appearances of spleen 53 differential diagnosis of splenic lymphoma 171–2 microscopic features 60 hilum, splenic 14 platelet phagocytosis 55 histiocytes, ceroid/sea-blue 128 see also ceroid histiocytes splenic atrophy 157 histiocytic medullary reticulosis 100 white pulp 53, 55–6

Index 186

IgA deficiency, selective 64–5 leishmaniasis 73, 175 immune response regulation 15 opportunistic 66 immunocompetence, systemic infections 70–4 post-splenectomy 11 immunocompromised patients systemic in immunocompetent individuals 70–4 autolysed tissue 161 inflammatory injury, spontaneous rupture 44 splenic atrophy 157 inflammatory pseudotumour see pseudotumour, immunocytoma 91–2 inflammatory immunodeficiency interdigitating reticulum cells 136 acquired due to chemotherapy/irradiation 65–6 investigations, ancillary 9 inherited syndromes 63–5 iron deposition see haemosiderin deposition see also acquired immune deficiency syndrome irradiation (AIDS); human immunodeficiency virus (HIV) acquired immunodeficiency 65–6 infection splenic atrophy 158–9 immunohistochemistry 9, 27–8, 29, 30–1, 123 isomerism, left-/right-sided 155 antibodies reactive with haemopoietic lineages 123 Kaposi’s sarcoma 70, 141 differential diagnosis aid 163 in Langerhans’ cell histiocytosis 137 Langerhans’ cell histiocytosis 136–7, 138 in systemic mastocytosis 119 cellular infiltrate 136, 137 red pulp 30–1, 32 diagnosis 137 white pulp 27–8, 29, 30 differential diagnosis 173, 175 see also individual subtypes of lymphoma peliosis 136 immunological deficit, hyposplenism 24 red pulp 173, 175 immunostaining 9 Langerhans’ cells 136–8, 175 immunosuppressive therapy 65–6 laparoscopic splenectomy 10–11 imprinting 9–10 lardaceous spleen 154 incidental splenectomy 5, 35–7 large B cell lymphoma 77, 78, 95–6, 99, 169 clinical information 37 large granular lymphocytes 57 marginal zones 39, 40 large granular lymphocytic leukaemia 97, 100, 101 normal appearance comparison 38 cytology 100 pathological changes 38 natural killer (NK) cell subtype 97, 100 post-traumatic spleen comparison 35–7 red pulp infiltration 169, 170 reactive changes 39–40 T cell subtype 97, 100, 170 red pulp plasmacytosis 39, 41 leishmaniasis 73 sinusoidal endothelium 39, 41 diffuse involvement of macrophages 173 spontaneous rupture 40 in AIDS 175 systematic analysis of sections 38–9 leukaemia infarction, splenic 145, 146, 147 see acute lymphoblastic leukaemia, acute myeloid appearance 145, 147 leukaemia B-prolymphocytic leukaemia; chronic causes 145 granulocytic leukaemia(CGL); chronic cystic change 153 lymphocytic leukaemia (CLL); hairy cell in malaria 72 leukaemia; large granular lymphocytic leukaemia; in portal hypertension 145 mast cell leukaemia; T cell prolymphocytic in sickle cell anaemia 50 leukaemia mechanisms 145 leukaemic transformation, acute, in chronic in systemic lupus erythematosus 59 myeloproliperative disorders 117 splenic atrophy 158 leukocytosis, post-splenectomy 25 infection lipid lamellar bodies in Niemann–Pick disease 130, 133 AIDS 68–70 lipid storage disorders, secondary 130 EBV 44, 70–1 lipopigment 131 Echinococcus 154 littoral cell angioma 140–1

187 Index

littoral cells 16 differential diagnosis 175–6 liver disease, portal vein obstruction 144 foamy 130, 135 lymph nodes, hilar 5, 8, 9, 171–2 Gaucher’s disease 127, 129 lymphadenopathy in FCCL 85 ITP 55, 58, 173 lymphangioma 139–40 Niemann–Pick disease 130, 131, 132 lymphatic channels 13 red pulp 175 lymphocyte traffic 22 storage 130, 132 lymphoid cells see B cells, T cells, lymphoma and plasma tingible body 28, 136 cells in storage disorders 175 lymphoid follicles see white pulp, nodules see also cordal macrophages lymphoid hyperplasia 153 macroscopic assessment 7 associated with rupture 36 malaria 71–3 focal 164 falciparum 74 white pulp 37 malignant histiocytosis 100 lymphoid malignancy 11 see also lymphoma mantle cell lymphoma (MCL) 77, 78, 81, 82–5, 86 lymphoid proliferation, neoplastic/reactive 163–4, 165, blastic variant 83 166–9, 170, 171–3 blastoid variant 166 germinal centres 164, 165, 166 cyclin D1 85, 86 hilar lymph nodes 171–2 cytology 83 mantle zones 166–7 distinction from B-CLL 83 marginal zones 167–8 dinstinction from FCCL 86 PALS 168–9 gastrointestinal tumours 82 red pulp 169, 170, 171 genetic features 85, 86 lymphoma 77–8, 163 germinal centres 164, 166 AIDS-associated 70 hilar lymph nodes 172 amyloidosis 155 immunophenotype 85 cystic nodules 153 mantle zones 166, 167, 168 diagnosis in autolysed tissue 160 marginal zone differentiation 83, 85 hilar lymph nodes in differential diagnosis neoplastic cells 83, 84, 85 171–2 white pulp involvement 83, 84 in immunocompromised patients 66 mantle zones 14, 15, 166–7, 168 leukaemic behaviour in red pulp 171 immunohistochemistry 28, 29 marginal zone differentiation 78 reactive changes 39 necrotic 152 marginal zone lymphoma (MZL) 2, 77, 78, 87, 90–1, 166 see also B cell lymphocytic lymphoma (B-CLL); B cell cytology 90 lymphoma; Burkitt’s lymphoma; follicle centre cell epithelioid granulomas 93, 173 lymphoma (FCCL); hepatosplenic ␥␦-T cell distinction from follicle centre cell lymphoma 86 lymphoma; lymphoplasmacytoid lymphoma; genetic features 91 mantle cell lymphoma (MCL); marginal zone hilar lymph nodes 172 lymphoma; non-Hodgkin’s lymphoma; splenic immunophenotype 91 lymphoma with villous lymphocytes (SLVL); T cell red pulp 93 lymphoma tumour cells 92 lymphomatous polyposis 82 see also mantle cell marginal zones 167–8 lymphoma B cells 15 lymphoplasmacytoid lymphoma 77, 78, 91–2, 94, 166 differentiation in lymphoma 78 genetic features 92 in follicle centre cell lymphoma 86 immunophenotype 92 in mantle cell lymphoma 83, 85 mantle zones 167 expansion 40 in HS 56, 59 macrophages in X-linked hyper-IgM syndrome 65 ceroid 134 functions 15

Index 188

marginal zones (cont.) megakaryocytes 115, 118 immunohistochemistry 28, 29 splenic atrophy 158 incidental splenectomy 36 spontaneous rupture 44 micro-anatomy 14, 15 systemic mastocytosis association 120 reactive changes 39, 42 see also extramedullary haemopoiesis (EMH) and red pulp interface 16 acute leukaemia T cells 15 myofibroblastic proliferation, inflammatory vaccination role 49 pseudotumour 139 mast cell leukaemia 120–1 mast cell tryptase 119, 122 needle biopsy 10 mast cells Neisseria meningitidis groups A and C vaccination 48 in myelodysplastic syndromes 120 nerve growth factor receptor (p75) 27 in systemic mastocytosis 119, 122 neutrophils in red pulp 113 mastocytosis see systemic mastocytosis Niemann–Pick disease 55, 126, 127, 128, 130–1 measles, mumps and rubella vaccination 19 cytoplasmic inclusions 130, 132–3 measurement of spleen 7, 8 diagnosis 130–1 megakaryocytes 111 differential diagnosis 176 clusters 115, 118, 119 foamy macrophages 130, 135 in autolysed tissue 160 lipid lamellar bodies 130, 133 in red pulp 113 sphingomyelinase deficiency 131 in myelofibrosis 118 variants 130, 131, 135 in myeloproliferative disorders 115, 118 non-Hodgkin’s lymphoma 52–3, 137 see also lymphoma in significant EMH 115, 116 cystic nodules 153 meningococci, vaccination 11 differential diagnosis 171 metastases 5, 151–2 spontaneous rupture 44 cystic 151, 152, 153 normoblastin, incidental EMH 112 differential diagnosis 152 molecular genetic analysis 9 oleogranuloma 125, 173, 175 mucosa-associated lymphoid tissue (MALT) 49, 90 in ITP 55 multifocal origin of spleen 17, 155 see also granulomas multiple myeloma 91 onion-skin fibrosis 59 multiple spleens 155–6 open circulation see circulatory pathways mycobacterial infection 66, 125–6 mycobacterial spindle cell pseudotumour 69 pancreas, ectopic splenic tissue 156 Mycobacterium avium-intracellulare 68–9, 126 Pappenheimer bodies 24 diffuse macrophage involvement 173 para-immunoblasts 78, 80, 81, 167 Mycobacterium tuberculosis 71 parasitic cysts 154 mycotic abscess 152 parenchymal haemorrhage 147 myelocytes, incidental EMH 112–13 pathologists, requirements 6–7 myelodysplastic syndromes 114 PAX-5 gene 92 features 120 peliosis 26, 141, 143–4 immunohistochemistry 123 causes 143 systemic mastocytosis association 120 cysts 143–4 myelofibrosis 2, 114, 115, 117 in Gaucher’s disease 127 megakaryocytes 118 in hairy cell leukaemia 173 myelopoiesis, transient abnormal (in Down’s syndrome) in Langerhans’ cell histiocytosis 136 121–2 megakaryocyte clusters 115, 118, 119 myeloproliferative disorders 18 spontaneous rupture 44 acute leukaemic transformation 117 penicillar arteries/arterioles 14 chronic 2, 114–15, 117, 178 peri-arteriolar lymphoid sheaths (PALS) 14–15 immunohistochemistry 123 antigen transport 22

189 Index

differential diagnosis 168–9 red pulp plasmacytosis 39, 41 immunohistochemistry 28, 30 sinusoidal endothelium 39, 41 in elderly patients 40 spontaneous rupture 40 in Epstein–Barr virus infection 70 systematic analysis of sections 38–9 in Hodgkin’s disease 104 processing of spleen tissue for histology 7–9 in immunodeficiency (including AIDS) 63–4, 68 proliferation centres in CLL 78 reactive changes 39 prolymphocytes see para-immunoblasts reticular meshwork 16–17 promyelocytes in incidental EMH 112–13 T cells 30 pseudo-Gaucher cells 128 T-independent antigens 23 pseudotumour, inflammatory 137, 139, 140 peri-follicular capillaries 16, 27 pyruvate kinase deficiency, red cells 52 peri-follicular zone 21 immunohistochemistry 30, 31 red cells reactive changes 39 auto-antibody coating 52 perisplenitis 159–60 cytoskeleton 52 phagocytosis destruction 47 in AIHA 54, 56 enzyme defects 51 intrasinusoidal of red cells 54–5 inclusions 24, 50 of platelets in ITP 55 intrasinusoidal phagocytosis 54–5 of sequestered material 21 membrane defects 24 physicians, requirements 5–6 pyruvate kinase deficiency 52 physiology of spleen, understanding 4 sequestration 21, 47, 51 plasma cells in Epstein–Barr virus infection 71 in HS 54 plasmacytosis 41 in malaria 72 in AIDS 67 sickle cell anaemia 50 in Felty’s syndrome 57 red pulp 14, 15–17 in ITP 55 autolysis 160 red pulp 42–3 in acute leukaemia 122 Plasmodium infection see also malaria 72 in AIDS 67 platelets in amyloidosis 154 auto-antibody coating 52 in B-CLL 78, 80 auto-immune destruction 67 CD8-positive T cells 67–8 phagocytosis in ITP 55 ceroid histiocytosis 131, 135 sequestration 51 circulatory pathways 19, 20 pneumococcal vaccination 6–7, 11 cordal macrophage disorders 173, 175 polyarteritis nodosa 44, 59 cords 30–1, 32 polycythaemia rubra vera (PRV) 114, 115, 117 reactive changes 39 post-splenectomy thrombocytosis 25 development 17, 18 polyposis, lymphomatous 82 differential diagnosis 168, 169, 170, 171 see also mantle cell lymphoma endothelial cells 41, 43 portal hypertension 144–5, 146 in Epstein–Barr virus infection 71 Gamna–Gandy bodies 147 erythrocytes in incidental EMH 112, 113–14 post-splenectomy infection 11 in FCCL 164, 171 post-traumatic spleen 35–7 Felty’s syndrome 61 clinical information 37 filtering areas 15–16 delayed rupture 37 functional correlates 19, 20, 21 incidental splenectomy comparison 35–7 in Gaucher’s disease 128, 129 marginal zones 39 granulomas 173, 175 normal appearance comparison 38 in hairy cell leukaemia 94, 174 pathological changes 38 hamartoma 153, 154 reactive changes 39–40 in hepatosplenic ␥␦T cell lymphoma 100, 102

Index 190

red pulp (cont.) sea-blue histiocytes 128 see ceroid histiocytes in hypersplenism 25 septicaemia 73–4 in hyposplenism 24 autolysis 160 immunohistochemistry 30–1, 32, 34 infarction 147 in Langerhans’ cell histiocytosis 173, 175 spontaneous rupture 43 in large granular lymphocytic leukaemia 57, 97, 101, sequestration 21, 26, 51 169, 170 causes 52 in lymphoma with leukaemic behaviour 171 platelets 51 in lymphoplasmacytoid lymphoma 92 red cells 47, 51 macrophages 175 in HS 54 in malaria 72 in malaria 72 in marginal zone lymphoma 90, 91, 93 severe combined immunodeficiency (SCID) syndrome microscopic abnormalities 54–5 63–4 in myelodysplastic syndromes 120 sickle cell disease 47, 49–51 non-filtering areas 16 splenic atrophy 158 normal 174 sinusoidal endothelium 16, 20, 31, 34 plasmacytosis 39, 41, 42–3 in HS 54 in portal hypertension 145, 146 in incidental splenectomy 39, 41 reactive changes 42–3 in post-traumatic spleen 39, 41 satellite nodules in SMZL 90, 93, 171 in reactive red pulp 41, 43 sequestration 26 sinusoids 16 sinusoids 16, 34 immunohistochemistry 31, 34 reactive changes 39 sickled red cells 50 in splenic atrophy 157 see also red pulp in storage disorders 127, 173, 175 size of spleen in elderly patients 39–40 structure 19, 20, 21 slicing technique 7, 8 in T cell prolymphocytic leukaemia 96 ␣-SMA see alpha-smooth muscle actin (␣-SMA) Reed–Sternberg cells 104, 105 see also Hodgkin’s disease small B cell lymphoma 77, 95 reservoir function of spleen 5 somatic hypermutation 23 reticulin 16, 174 see also lymphoma reticulum cells, interdigitating 136 spectrin 52 rhabdomyosarcoma 141 spherocytosis Rhesus antigens 52 thalassaemia 51 Rochalimaea henselae see Bartonella henselae see also hereditary spherocytosis (HS) rodent spleen 5 sphingomyelinase deficiency 131 marginal zone capillary network 16 spindle cell tumours, malignant 140–1 rodents, haemopoiesis 18 spindle cells rupture 1 in mast cell leukaemia 120 anti-coagulant associated 143 ␣-SMA expression 21, 30, 32 delayed 37 in systemic mastocytosis 119 lymphoid hyperplasia 36 splenectomy 1 rupture, spontaneous 40, 43–4 in AIHA 51–2 causes 44 diagnostic 2–3, 6 in Epstein–Barr virus infection 70 EMH 112, 117 in malaria 72 in hairy cell leukaemia 94 in peliosis 141 for hamartoma 152–3 histological material 3 sago spleen 154 in HS 51 sarcoidosis 125–6 inflammatory pseudotumour 137 satellite nodules in SMZL 90, 93, 171 in ITP 51–2 sea-blue histiocyte syndrome see ceroid histiocytosis laparoscopic 10–11

191 Index

leukocytosis 25 endothelial cells 127 partial 11, 14 primary 135–6, 176 patient implications 11–12 red pulp 173, 175 in polycythaemia rubra 25 secondary 135–6, 176 purpose 6 tingible body macrophages 175 therapeutic 1–2, 5–6 storage macrophages 130, 132 thrombocytosis after 25 Streptococcus pneumoniae 69 vaccination 48–9 vaccination 48 see also incidental splenectomy subcapsular tissue fixation 9 splenic artery 13–14 sudden infant death syndrome 18 splenic cords 16 sugar-icing of spleen 8, 159–60 in hairy cell leukaemia 95, 98 surface immunoglobulin M (SIgM) 81, 82 in reactive red pulp 43 surgeons, requirements 5–6 in sickled red cells 50 systemic lupus erythematosus 44, 57, 59 see also red pulp systemic mastocytosis 117, 119, 120, 121, 122 splenic function loss 11–12 immunohistochemistry 119, 122 see also hyposplenism mast cell leukaemia 120–1 splenic hamartoma see hamartoma mast cells 119, 122 splenic lymphoma with villous lymphocytes (SLVL) 2, myelodysplastic syndrome association 120 90–1 myeloproliferative disorder association 120 epithelioid granulomas 173 hilar lymph nodes 172 T cell lymphoma 77 splenic marginal zone lymphoma see marginal zone hepatosplenic (␥␦) 100–1, 102–3 lymphoma (MZL) high grade 77, 78, 96 splenic tissue spillage 6 PALS 168 splenic vein 13–14 peripheral of CD8-positive cells 169 splenic–gonadal fusion 156 pleomorphic 96 splenomegaly 6, 25, 26 with erythrophagocytosis 100, 102 in amyloidosis 154 T cell prolymphocytic leukaemia (T PLL) 96–7, 99, 168–9 extreme 43 cytology 99 in Felty’s syndrome 57 genetic features 97 in hairy cell leukaemia 94 immunophenotype 96–7 hypersplenism 47 T cells 15 in Langerhans’ cell histiocytosis 136 inherited immunodeficiency states 65 in lymphoplasmacytoid lymphoma 92 T-independent antigens 23 in malaria 71–2 target cells 24 in mantle cell lymphoma 82–3 thalassaemias 47, 49–51, 50–1 portal hypertension 144 thorium dioxide (Thorotrast) 141, 158–9 sickled cell sequestration 50 thrombocytopenia, HIV-associated 11 significant EMH 114 thrombocytopenic purpura see idiopathic in storage disorders 126 thrombocytopenic purpura (ITP) in tuberculosis 71 thrombocytosis post-splenectomy 25 see also tropical splenomegaly syndrome tingible body macrophages 28, 136 splenosis 6, 156–7 in storage disorders 175 splenunculi 156–7 trabeculae, connective tissue 14 staining for histology 9 transient abnormal myelopoiesis (in Down’s syndrome) steroid therapy see corticosteroids 121–2 storage disorders trauma 4, 6 ceroid deposition 175–6 trisomy 8q 97 cordal macrophages 26, 126–8, 129, 130–1, 132–4, trisomy 12 in B-CLL 81, 82 135–6 trisomy 13, ectopic splenic tissue 156

Index 192

trisomy 21 in transient abnormal myelopoiesis 121–2 extra-follicular focus 22–3 tropical splenomegaly syndrome 72–3 in FCCL 164, 165 tuberculosis 68, 69, 71, 126 see also mycobacterial function 22–3 infection granulomas 173, 175 abscess 152 in hepatosplenic ␥␦T cell lymphoma 100 peliosis 143 in HIV infection 67 in Hodgkin’s disease 104, 105 ulcerative colitis 158 in HS 56, 59 immunohistochemistry 27–8, 29, 30 vaccination 6–7, 11, 19 incidental splenectomy 36 morphological changes 7, 48 involution 42, 43 post-operative 48–9 in ITP 53 pre-splenectomy 48–9 lymphoid hyperplasia 37 vascular supply of spleen 13–14 in lymphoplasmacytoid lymphoma 92 segmental 14 in mantle cell lymphoma 83, 84 vascular tumours 26 see also angioma, angiomatosis, mantle zones 28, 166 angiosarcoma, littoral cell angioma, in marginal zone lymphoma 90, 91 lymphangioma marginal zones 28, 29 vasculitis 59 microscopic abnormalities 55–6 villous lymphocytes 87, 90 nodules 14, 15 diﬀerential diagnosis 168 Waldenström’s macroglobulinaemia 92, 94 in Epstein–Barr virus infection 71 Waldeyer’s ring tumours 82 in FCCL 86, 88 weight of spleen 38 in malaria 72 vaccine-related changes 48 oleogranuloma 125 white pulp 6, 14 in tuberculosis 71 activity 18–19 peri-follicular zone 30, 31 in AIDS 66 in post-traumatic spleen 36 in amyloidosis 154 reactive changes 40, 42 atrophy 42, 64, 157 reactive follicle 165 in HIV infection 68 scaﬀold 21 autolysis 160 in storage disorders 127 in B-CLL 78, 79 structure 22–3 in Bruton’s agammaglobulinaemia 65 in systemic lupus erythematosus 59 in cordal macrophage disorders 173, 175 development 17 X-linked hyper-IgM syndrome 65