Characteristically only the urine voided at night and in the morning on waking is dark in colour.
Rx with eculizumab.
Causes of MAHA
When fragmentation of red cells occurs in an abnormal microcirculation.
The Peripheral Blood film
is variation in RBC size, eg megaloblastic anaemia, thalassaemia, IDA.
RBCs show many spicules due to an unstable red cell membrane lipid structure (eg in abetalipoproteinaemia).
Basophilic RBC stippling:
Denatured RNA found in RBCs, indicating accelerated erythropoiesis or defective Hb synthesis. Seen in lead poisoning, megaloblastic anaemia, myelodysplasia, liver disease, haemoglobinopathy eg thalassaemia.
Nucleated precursor cells. They are not normally in peripheral blood, but are seen in myelofibrosis, leukaemia or malignant infiltration by carcinoma.
Irregularly shaped cells occurring in uraemia.
Two populations of red cells. Seen after treatment of Fe, B 12 or folate deficiency, post-transfusion, or with primary sideroblastic anaemia, where a clone of abnormal erythroblasts produce abnormal red cells, alongside normal red cell production.
DNA nuclear remnants in RBCs, which are normally removed by the spleen. Seen post-splenectomy and in hyposplenism (eg sickle cell disease, coeliac disease, congenital, UC/Crohn's, myeloproliferative disease, amyloid). Also in dyserythopoietic states: myelodysplasia, megaloblastic anaemia.
Less dense staining of RBCs due to reduced Hb synthesis, seen in IDA, thalassaemia, and sideroblastic anaemia (iron stores unusable).
Immature neutrophils are sent out of the marrow, eg in infection.
Immature cells (myelocytes, promyelocytes, metamyelocytes, normoblasts) seen in film. Due to marrow infiltration (eg malignancy) when these cells are displaced; also seen in anorexia, sepsis, severe haemolysis.
A marked leucocytosis (WCC>50). Seen in severe illness eg with infection or burns, and also in leukaemia.
Granules of siderocytes containing iron. Seen in lead poisoning, carcinomatosis, and post-splenectomy.
is variation in RBC shape, eg in IDA, myelofibrosis, thalassaemia.
RBCs of different ages stain unevenly (young are bluer). This is a response to bleeding, haematinic replacement (ferrous sulfate, B 12 , folate), haemolysis, or marrow infiltration. Reticulocyte count is raised.
(normal range: 0.8-2%). Young, larger RBCs (contain RNA) signifying active erythropoiesis. Increased in haemolysis, haemorrhage, and if B 12 , iron or folate is given to marrow that lack these.
Hypermature white cells: hypersegmented polymorphs (>5 lobes to nucleus) seen in megaloblastic anaemia, uraemia, and liver disease.
Red cells stack on each other (it causes a raised ESR). Seen with chronic inflammation, paraproteinaemia and myeloma.
Spherical cells found in hereditary spherocytosis and autoimmune haemolytic anaemia.
Fragmented RBCs sliced by fibrin bands, in intravascular haemolysis. Look for microangiopathic anaemia, eg DIC , haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura or pre-eclampsia.
(also known as Mexican hat cells). These are RBCs with central staining, a ring of pallor, and an outer rim of staining seen in liver disease, hyposplenism, thalassaemia and, in small numbers, in iron-deficiency anaemia.
A normal blood film, with a neutrophil, normal red cells, and platelets (arrows).
Microcytic hypochromic cells in iron deficiency anaemia
Poikilocytosis and anisocytosis seen in iron deficiency anaemia.
Pathological ring sideroblasts in the bone marrow, with a perinuclear ring of iron granules, found in sideroblastic anaemia
Left-shift: presence of immature neutrophils in the blood
Reticulocytes. RNA in RBCs; supravital staining (azure B; cresyl blue) is needed
Film in hyposplenism: target cell (short arrow), acanthocyte (long arrow) and a Howell-Jolly body (arrow head).
A Cabot ring; these red/purple-staining filamentous figure-of-8 rings are often seen in RBCs with basophilic stippling. They occur in severe or megaloblastic anaemia, leukaemia, and lead poisoning.
Megaloblastic anaemia: peripheral blood film showing many macrocytes and one hypersegmented neutrophil (normally there should be <5 segments).
A bite cell in G6PD, following removal of Heinz bodies by the spleen. Heinz bodies are formed from oxidized, denatured Hb during oxidative crises
Blister cells (arrows) in G6PD, following removal of Heinz bodies. Also contracted red cells (arrowheads).
Microangiopathic anaemia eg from DIC: numerous cell fragments (schistocytes) are present
Fibrin strands, deposited in HUS & TTP, slice up passing red cells (microangiopathic anaemia).
Blood film in sickle-cell anaemia: there are sickle cells, target cells, and a nucleated red cell .