Bleeding tendencies in children

Dr Wrishi Raphael

Rahim, a 3 year old boy hailing from Manikgonj presented in Paediatric OPD of Dhaka Shishu Hospital with his mother who complained that Rahim had fine bruising over his trunk and limbs with a fine rash over his body. On their way by bus from Manikgonj, Rahim had a nose bleed which lasted for twenty minutes.
The rash looked like small red or purple spots which in medical terms fits the description of petechial rash. On further exam there were no alarming features like enlargement of any of the lymph vessels or organs. The child was otherwise fine; he had no respiratory distress, fever or any of the other indicators of severe, life threatening disease.

Bleeding tendencies in children are very common world over and general practitioners everywhere should be able to differentiate between the common ones before referring. By the phrase common ones, the aim should be to discern between the following; Von Willebrand disease, Henoch Schonlein Purpura, Immune Thrombocytopenic Purpura [ITP], Haemophillia and Leukemia.

Rahim’s petechial rash indicates that there is bleeding into the skin because of a disease of platelets or blood vessels. Petechial rash do not occur in Haemophilia and Von Willebrand Disease [vWD] so Rahim is not suffering from either. Since bleeding disorders like von Willebrand and Haemophilia appear soon after birth and they are closely related to heavy bleeding after minor trauma, Rahim’s medical history shows no association with excessive bleeding episodes after circumcision or other injuries.

A positive family history can be a positive pointer to the diagnosis of vWD or Haemophilia:
Sex-linked recessive pattern: haemophilia A or B
Autosomal dominant pattern: vWD
Autosomal recessive pattern: deficiency of coagulation factors V, VII and X.

Henoch Schonlein Purpura (HSP) has a particular distribution of rash over the extensor surface of thigh and buttocks and is associated with joint pain and abdominal symptoms. Children with HSP also suffer kidney diseases which clinically resemble glomerulonephritis.

A child with acute leukaemia is usually ill for several weeks with a wide variety of symptoms. Eventually it becomes obvious that the child has bone marrow disorder evidenced by bruising and pallor. Enlargement of liver, spleen and lymph glands are also more common in leukaemia then other disorders.

A normal response to previous coagulation stresses (e.g. dental extraction, circumcision or pregnancy) indicates an acquired problem. If acquired, evidence can then be checked for malignancy, infection, liver disease and drugs.

Some viral illnesses especially Dengue, are capable of producing rash but such children are usually ill for a week or more and the rash usually always disappears with the illness.

But Rahim however, did not have such episodes of illness in the last six years. In the absence of other features the general practitioner may confidently infer that the child has Immune Thrombocytopenic Purpura. Although platelet abnormalities may occasionally present as early bleeding following trauma; coagulation factor deficiencies present with delayed bleeding after initial haemostasis is achieved by normal platelets.

The laboratory checklist for children who may present with a spectrum of such problems are given below, provided reliable facilities exist in the patient’s vicinity:  
Full Blood Count
Platelet Count
Prothrombin time
Activated Partial Thromboplastin Time [APTT]
In order to establish or differentiate between von Willebrand’s disease and Haemophilia one will need to perform one or all of the following:
Factor VIII.
vW factor activity.
vW factor antigen.

All of the diseases briefly discussed require thorough history taking, exhaustive clinical examination and modern laboratory equipment but general practitioners can easily make educated guesses and about the patients predicament if their skills and vigilance can be
incorporated with patience and delicacy. (Reprint)

 

Bleeding tendencies in children

Dr Wrishi Raphael Rahim, a 3 year old boy hailing from Manikgonj presented in Paediatric OPD of Dhaka Shishu Hospital with his mother who complained that Rahim had fine bruising over his trunk and limbs with a fine rash over his body. On their way by bus from Manikgonj, Rahim had a nose bleed which lasted for twenty minutes. The rash looked like small red or purple spots which in medical terms fits the description of petechial rash. On further exam there were no alarming features like enlargement of any of the lymph vessels or organs. The child was otherwise fine; he had no respiratory distress, fever or any of the other indicators of severe, life threatening disease. Bleeding tendencies in children are very common world over and general practitioners everywhere should be able to differentiate between the common ones before referring. By the phrase common ones, the aim should be to discern between the following; Von Willebrand disease, Henoch Schonlein Purpura, Immune Thrombocytopenic Purpura [ITP], Haemophillia and Leukemia. Rahims petechial rash indicates that there is bleeding into the skin because of a disease of platelets or blood vessels. Petechial rash do not occur in Haemophilia and Von Willebrand Disease [vWD] so Rahim is not suffering from either. Since bleeding disorders like von Willebrand and Haemophilia appear soon after birth and they are closely related to heavy bleeding after minor trauma, Rahims medical history shows no association with excessive bleeding episodes after circumcision or other injuries. A positive family history can be a positive pointer to the diagnosis of vWD or Haemophilia: Sex-linked recessive pattern: haemophilia A or B Autosomal dominant pattern: vWD Autosomal recessive pattern: deficiency of coagulation factors V, VII and X. Henoch Schonlein Purpura (HSP) has a particular distribution of rash over the extensor surface of thigh and buttocks and is associated with joint pain and abdominal symptoms. Children with HSP also suffer kidney diseases which clinically resemble glomerulonephritis. A child with acute leukaemia is usually ill for several weeks with a wide variety of symptoms. Eventually it becomes obvious that the child has bone marrow disorder evidenced by bruising and pallor. Enlargement of liver, spleen and lymph glands are also more common in leukaemia then other disorders. A normal response to previous coagulation stresses (e.g. dental extraction, circumcision or pregnancy) indicates an acquired problem. If acquired, evidence can then be checked for malignancy, infection, liver disease and drugs. Some viral illnesses especially Dengue, are capable of producing rash but such children are usually ill for a week or more and the rash usually always disappears with the illness. But Rahim however, did not have such episodes of illness in the last six years. In the absence of other features the general practitioner may confidently infer that the child has Immune Thrombocytopenic Purpura. Although platelet abnormalities may occasionally present as early bleeding following trauma; coagulation factor deficiencies present with delayed bleeding after initial haemostasis is achieved by normal platelets. The laboratory checklist for children who may present with a spectrum of such problems are given below, provided reliable facilities exist in the patients vicinity: Full Blood Count Platelet Count Prothrombin time Activated Partial Thromboplastin Time [APTT] In order to establish or differentiate between von Willebrands disease and Haemophilia one will need to perform one or all of the following: Factor VIII. vW factor activity. vW factor antigen. All of the diseases briefly discussed require thorough history taking, exhaustive clinical examination and modern laboratory equipment but general practitioners can easily make educated guesses and about the patients predicament if their skills and vigilance can be incorporated with patience and delicacy. (Reprint)