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The major forms of benign hematologic conditions are anemia, neutropenia, transfusion reactions, Gaucher disease, and porphyria. Anemia is a sign of disease rather than a disease itself. Anemia results from 1 or more of 3 pathologic mechanisms: inadequate production of red blood cells (RBCs) by the bone marrow, blood loss, or premature destruction of RBCs. The major causes of neutropenia include hematologic neoplasm, metastatic neoplasm involving the marrow, irradiation, vitamin B12 deficiency and folate deficiency, drugs, infections, congenital or acquired primary disorders of hematopoiesis, autoimmune neutropenia, hypersplenism, hemodilution, and benign idiopathic neutropenia. The porphyrias are enzyme disorders that are autosomal dominant with low disease penetrance, except for congenital erythropoietic porphyria, which is autosomal recessive, and porphyria cutanea tarda, which may be acquired and is associated with hepatitis C and hemochromatosis. ...Less

The coagulation system has 2 essential functions: to maintain hemostasis and to prevent and limit thrombosis. The procoagulant component of the hemostatic system prevents and controls hemorrhage. Vascular injury results in activation of hemostasis, which consists of vasospasm, platelet plug formation (platelet activation, adhesion, and aggregation), and fibrin clot formation (by activation of coagulation factors in the procoagulant system). The anticoagulant system prevents excessive formation of blood clots, and the fibrinolytic system breaks down and remodels blood clots. Quantitative abnormalities (deficiencies) and qualitative abnormalities of platelets and coagulation factors lead to bleeding disorders, whereas deficiencies of the anticoagulant system are risk factors for thrombosis. Common disorders of hemostasis and thrombosis are reviewed. ...Less

Jonathan Purday Anaemia  - Sickle cell disease  - Porphyria  - Rare blood disorders  - Coagulation disorders  - Haemophilia and related clotting disorders  - Thrombocytopenia  - Anticoagulants  - Antiplatelet drugs  - Fibrinolytics  - Antifibrinolytics/haemostatic drug therapy  - Haematological management of the bleeding patient  - Hypercoagulability syndromes  ...Less

Clotting derangement - Haemoglobin transfusion triggers - Heparin-induced thrombocytopaenia - Disseminated intravascular coagulation - Neutropenia - Severe haemolysis - Vasculitic crises ...

Heparin and protamine - Preoperative antithrombotic drugs - Bleeding and transfusion - The postoperative bleeding patient - Management of patients requiring postoperative anticoagulation - Further reading ...Less

Anaemia - Sickle cell disease - Acute splenic sequestration - Neutropenia - Platelet disorders - Disorders of coagulation - Anticoagulants and antithrombotics - Product transfusions - Further reading ...Less

Full blood count and ESR - Anaemia: diagnosis and initial investigation - Iron deficiency anaemia - Haemolysis and aplastic anaemia - Haemoglobinopathy - Bleeding and clotting disorders - Anticoagulation - Haematological malignancy - Acute leukaemia - Chronic leukaemia and myeloproliferation - Lymphoma - Immune deficiency syndromes - Allergies ...Less

The hematologic neoplasms include lymphoproliferative disorders (eg, chronic lymphocytic leukemia [CLL]/small lymphocytic lymphoma [SLL], large granular lymphocyte leukemia, hairy cell leukemia [HCL], Hodgkin lymphoma, non-Hodgkin lymphoma), plasma cell disorders (multiple myeloma, light chain amyloidosis, Waldenström macroglobulinemia, POEMS syndrome, heavy chain disease, plasmacytoma), chronic myeloid neoplasms (chronic myeloid leukemia, the BCR/ABL-negative myeloproliferative neoplasms, myelodysplastic syndromes), and acute leukemia (acute myeloid leukemia, acute lymphocytic leukemia). In addition, clonal but not overtly malignant conditions are common in the general population, including monoclonal gammopathy of undetermined significance (MGUS) and monoclonal B lymphocytosis (MBL). ...Less