Haematological Presentations Flashcards

Question

NEUTROPENIA | Pancytopenia DDX

Answer 1

• Bone marrow depression, e.g. cytotoxic agents, malignant infiltration • Severe B12 or folate deficiency • Hypersplenism

Answer 2

``` Physiological • Healthy black individuals Infection • Overwhelming sepsis, e.g. septicaemia • Brucellosis • TB • Typhoid Immune • SLE • Felty’s syndrome Autoimmune neutropenia Drug induced, e.g. indomethacin, chloramphenicol, co-trimoxazole ```

Answer 3

* Acute infection * Trauma * Surgery * Radiotherapy * Chemotherapy * Steroid therapy * SLE * Uraemia * Cushing’s syndrome * Hodgkin’s disease * Immunodeficiency syndromes, e.g. HIV

Answer 4

``` ■■ FBC Hb  in malignancy, bone marrow depression. WCC  – type of white cell involved. ■■ U&Es Uraemia. Renal involvement, e.g. SLE. ■■ Mantoux test TB. ■■ Antibody screen SLE. ■■ Antineutrophil antibody screen Autoimmune neutropenia. ■■ Serum B12 and folate B12 and folate deficiency. ■■ Rheumatoid factor Felty’s disease. ■■ Viral titres HIV. ■■ Bone marrow Bone marrow depression. ```

Answer 5

increase in red cell concentration above the normal limit, usually accompanied by a corresponding increase in haematocrit and haemoglobin concentration. Polycythaemia can be ‘real’ due to a true increase in red cell concentration or ‘apparent’, due to low plasma volume (e.g. dehydration). Primary polycythaemia (polycythaemia vera) is due to a neoplasm of the myeloid series and secondary polycythaemia is due to raised erythropoietin levels and may be appropriate (chronic low oxygenation) or inappropriate (erythropoietin production from renal tumours).

Answer 6

Primary polycythaemia • Polycythaemia vera Secondary polycythaemia • Appropriate erythropoietin production (hypoxia) • Smoking (due to carbon monoxide exposure) • Chronic respiratory disease • Long-term exposure to high altitude • Cyanotic heart disease • High-affinity haemoglobinopathy ``` Inappropriate erythropoietin production • Renal carcinoma or cysts • Renal artery stenosis • Renal amyloidosis • Hepatocellular carcinoma • Massive uterine fibroids • Cerebellar haemangioblastoma ```

Answer 7

* Low plasma volume polycythaemia * Dehydration * Burns * Enteropathy * Gaisböck’s syndrome

Answer 8

■■ FBC Hb 17.5 g/dL in men and 15.5 g/dL in women with accompanying rise in red cell count. Haematocrit more than 55% in men and 47% in women. Thrombocytosis and neutrophil leucocytosis present in up to 50% of cases of polycythaemia vera. ■■ Blood film Occasional metamyelocytes and myelocytes on blood film and polycythaemia vera. ■■ Serum uric acid  uric acid in polycythaemia vera due to increased cell turnover. ■■ ABG O2 concentration in blood normal in primary polycythaemia but reduced in secondary polycythaemia due to respiratory failure or cardiac disease with right to left shunt. ■■ Total red cell volume Red cells in a sample of blood from the patient are tagged with 51Cr, and the labelled red cells are re-injected. The dilution of the isotope in a subsequent venous blood sample is used to calculate the red cell mass. The normal range is 25–35 mL/kg in men and 22–32 mL/kg in women. In the presence of ongoing blood loss (there is a predisposition to bleeding and peptic ulceration), iron-deficient polycythaemia results and the haemoglobin and haematocrit may be normal or low. The condition should be suspected when there are obviously iron-deficient red cells (low MCV) without anaemia and with a very high red cell count. ■■ Plasma volume 125I-albumin is used to estimate total plasma volume, the normal range is 35–45 mL/kg. This is usually performed in conjunction with the estimation of total red cell volume to distinguish between real and apparent polycythaemia (the latter is due to low plasma volume). ■■ Bone marrow aspiration In polycythaemia vera there is a hypercellular bone marrow with erythroid hyperplasia. Megakaryocytes may be prominent and increased reticulin deposition is common.

Answer 9

150 × 109/L. The causes of a low platelet count can be grouped according to reduced production, decreased survival or sequestration in the spleen.

Answer 10

``` • Aplastic anaemia • Drugs, e.g. tolbutamide, alcohol, cytotoxic agents • Viral infections, e.g. EBV, CMV • Myelodysplasia • Bone marrow infiltration, e.g. carcinoma, leukaemia, myeloma, myelofibrosis • Megaloblastic anaemia • Hereditary thrombocytopenia ```

Answer 11

``` IMMUNE • ITP • Drugs, e.g. heparin, quinine, sulphonamides, penicillins, gold • Infections • Post-transfusion ``` NON-IMMUNE • Disseminated intravascular coagulation (DIC) • Thrombotic thrombocytopenic purpura • Haemolytic uraemic syndrome

Answer 12

■■ FBC Isolated thrombocytopenia likely to be due to increased destruction. Thrombocytopenia due to marrow failure likely to be associated with anaemia, leucopenia. ■■ Blood film Red cell fragmentation (DIC). Platelet size (large in ITP and some hereditary conditions). WBC (atypical lymphocytes/lymphoblasts). ■■ Bleeding time Prolonged if platelet count <80 × 109/L. ■■ Coagulation screen Clotting times normal in autoimmune thrombocytopenia but may be abnormal in other causes, e.g. DIC. ■■ Platelet function test Associated abnormal platelet function, e.g. uraemia. ■■ Serology Antinuclear antibodies (autoimmune disease). Monospot (glandular fever). Antiplatelet antibodies (unreliable). Paul–Bunnell test (glandular fever). CMV serology. ■■ U&Es, creatinine Haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura. ■■ LFTs Hepatitis, e.g. due to EBV, CMV. ■■ Bone marrow Megakaryocytes increased in number and size in ITP. Absence or reduction in megakaryocytes rules out ITP. Aplastic anaemia. Leukaemia. Marrow infiltration (carcinoma, lymphoma, myeloma).

Answer 13

``` • Haemorrhage • Haemolysis • Trauma • Surgery • Post-partum • Chronic inflammation, e.g. rheumatoid arthritis, inflammatory bowel disease • Iron deficiency anaemia • Hyposplenism (splenectomy, splenic atrophy) ```

Answer 14

■■ FBC Platelets . Hb  (iron deficiency anaemia). Hb  (polycythaemia rubrum vera). MCV  (iron deficiency anaemia). PCV  (polycythaemia rubrum vera). WBC  (infection, chronic myeloid leukaemia). ■■ Blood film Microcytic hypochromic anaemia in iron deficiency. Thrombocytosis, giant platelets, platelet clumping, megakaryocyte fragments in essential thrombocythaemia. ■■ ESR Chronic inflammation. Malignancy. ESR very high in myeloma. ■■ Serum iron and TIBC Serum iron  TIBC  in iron deficiency. ■■ Serum ferritin Reduced in iron deficiency. Not affected by chronic disease. ■■ Bone marrow Essential thrombocythaemia – hypercellularity, increased megakaryocytes, large megakaryocytes. Polycythaemia rubrum vera – increased cellularity due to hyperplasia for erythropoietic cells, granulocytopoietic cells and megakaryocytes. Chronic myeloid leukaemia – marked hypercellularity due to myeloid hyperplasia. Myelofibrosis – ‘dry tap’ (aspiration unsuccessful).

Answer 15

* Essential thrombocythaemia (myeloproliferative disorder) | * Other chronic myeloproliferative disorders, e.g. chronic myeloid leukaemia, myelofibrosis, polycythaemia rubrum vera.

Haematological Presentations Flashcards

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