A 36-year-old woman presents to her general practitioner (GP) complaining of spontaneous bruising mainly on her legs

history
A 36-year-old woman presents to her general practitioner (GP) complaining of spontaneous bruising mainly on her legs. The bruising has been noticeable over the last 4–6 weeks. She cannot remember any episodes of trauma. In addition, her last two menstrual periods have been abnormally heavy, and she has suffered a major nosebleed. She otherwise feels well and is working full time as a secretary. There is no significant past medical history. She is married with one daughter aged 11 years. There is no family history of
a bleeding disorder. She is a non-smoker and drinks a small amount of alcohol socially.
Examination
On examination, there are multiple areas of purpura on her legs and to a lesser extent on
her abdomen and arms. The purpuric lesions vary in color from black–purple to yellow.
There are no signs of anemia, but there are two bullae in the mouth and there is spontaneous bleeding from the gums. There are no retinal hemorrhages on funduscopy. Blood
pressure is 118/72 mmHg. Examination of the cardiovascular, respiratory and abdominal
systems is unremarkable.

Questions
• What is the likely diagnosis?
• How would you further investigate and manage this patient?

Question answer:

Diagnosis:

·         This woman has spontaneous bruising due

·         to idiopathic thrombocytopenic purpura (ITP).

·         She has profound thrombocytopenia with a platelet count of 4 % 109 /L.

·         An increased tendency to bleed or bruise can be due either to platelet, coagulation, or blood vessel abnormalities.

·         Platelet/vessel wall defects cause spontaneous purpura in the skin and mucous membranes or immediately after trauma.

·         Coagulation defects cause hematomas and haemarthroses usually with a time delay after trauma.

·         Positive family history or early onset of bleeding suggests hemophilia.

·         The distribution of bruising may suggest the diagnosis.

·         Thrombocytopenic purpura is most evident over the ankles and pressure areas.

·         Retinal hemorrhages tend to occur if there is a combination of severe thrombocytopenia and anemia.

·         Senile purpura and steroid-induced bruising occur mainly on the forearms and backs of the hands.

·         Henoch–Schoenlein purpura typically occurs over the extensor aspects of the limbs and buttocks.

·         Scurvy causes bleeding from the gums and around the hair follicles.

·         Non-accidental injury in children can present with bruising. ITP usually occurs in young and middle-aged women.

·         In addition to the purpuric lesions in the skin there may be menorrhagia, epistaxis or occult or overt gastrointestinal hemorrhage.

·         In this woman’s case, there is a mild normochromic normocytic anemia due to recent blood loss.

·         Splenomegaly is usually absent.

Futher investigation and management of patients:

The causes of thrombocytopenia can be divided into disorders of reduced production of platelets or decreased survival of platelets.

·         Decreased production of platelets can be due to marrow infiltration, for example by leukemia or malignancy, or as a result of toxins, for example, alcohol, drugs (e.g. chemotherapy), radiation or viruses (e.g. cytomegalovirus [CMV]).

·         Platelet survival is reduced in ITP due to antibodies directed against the platelets. Secondary causes of ITP include systemic lupus erythematosus (SLE), lymphoma, drugs such as quinine, heparin , and alpha-methyldopa, and hypersplenism. Platelet consumption is increased in disseminated intravascular coagulation and thrombotic thrombocytopenic purpura (TTP).

Management of patients;

The patient should be immediately referred to a hematology unit. Platelet transfusion is usually given if there is significant bleeding or the platelet count is less than 15 % 109 /L to prevent a major spontaneous bleed. Investigations include assaying for platelet-associated immunoglobulin G (IgG), excluding other causes of thrombocytopenia such as SLE and performing a bone marrow aspirate. In this case, it will show increased numbers of megakaryocytes consistent with increased platelet turnover. The platelet count in ITP normally will rise rapidly after the commencement of either corticosteroids or intravenous immunoglobulin. The disease often runs a remitting and relapsing course. Splenectomy may be necessary