Management of Refractory Platelet Transfusions

Patients who do not respond to platelet transfusions should be evaluated to ensure that their platelet counts are increasing adequately. The best way to do this is by taking a blood sample one or two hours after the transfusion. If the patient’s platelet counts are still low, further investigation is necessary. However, it is not appropriate to request HLA-matched platelets or a directed platelet donation at this stage.

Continuing to give random platelet transfusions is also not recommended. Platelets are necessary for the patient’s recovery, but it is important to determine why the transfusions are not working. Therefore, checking for HLA antibodies is the next step in the management of refractory platelet transfusions. Once the cause of the refractory response is identified, appropriate treatment can be initiated.

In summary, managing refractory platelet transfusions involves evaluating the patient’s response to the transfusions, checking for HLA antibodies, and determining the underlying cause of the refractory response. Platelets are still necessary for the patient’s recovery, but it is important to address the underlying issue to ensure that the transfusions are effective.

Treatment Options for Non-Haemolytic Febrile Transfusion Reaction

Non-haemolytic febrile transfusion reaction is a common acute reaction to plasma proteins during blood transfusions. If a patient experiences this reaction, the transfusion should be temporarily stopped, and clerical checks should be repeated. The patient should be treated with paracetamol, and observations should be repeated more regularly (every 15 minutes).

If the patient’s temperature is less than 38.5 degrees, and they are asymptomatic with normal observations, the transfusion can be continued with more frequent observations and paracetamol. However, if the patient experiences transfusion-associated circulatory overload, furosemide is a suitable treatment option.

Adrenaline is not needed unless there are signs of anaphylaxis, and antihistamines are only suitable for urticaria during blood transfusions. Therefore, it is essential to identify the specific type of transfusion reaction and provide appropriate treatment accordingly.

Partial remission occurs when a patient meets all the criteria for complete remission except for having more than 5% bone marrow blasts. To be diagnosed with partial remission, the blast cells can be between 5% and 25% and must have decreased by at least 50% from their levels before treatment.

Complete remission is achieved when a patient meets specific criteria, including having a neutrophil count of over 1.0 × 109/l and a platelet count of over 100 × 109/l, not requiring red cell transfusions, having normal cellular components on bone marrow biopsy, having less than 5% blasts in the bone marrow without Auer rods present, and having no signs of leukemia anywhere else in the body.

Complete remission with incomplete recovery is when a patient meets all the criteria for complete remission except for continuing to have neutropenia or thrombocytopenia.

Resistant disease occurs when a patient fails to achieve complete or partial remission and still has leukemia cells in their peripheral blood or bone marrow seven days after completing initial therapy.

A morphologic leukemia-free state is when a patient has less than 5% bone marrow blasts without blasts with Auer rods present and no extramedullary disease, but they do not meet the criteria for neutrophils, platelets, and blood transfusions.

Management of Unexplained Microcytic Anemia with Low Ferritin

Unexplained microcytic anemia with low ferritin levels requires prompt investigation to identify the underlying cause. According to National Institute for Health and Care Excellence (NICE) guidelines, men with unexplained iron deficiency anemia and a hemoglobin level below 110 g/l should be urgently referred for upper and lower gastrointestinal investigations, regardless of age. A trial of oral iron may be appropriate in pregnant women or premenopausal women with a history of menorrhagia and without gastrointestinal symptoms or a family history of gastrointestinal cancer.

A faecal occult blood test is not recommended as it has poor sensitivity and specificity. Referral to haematology is not necessary as first-line investigations would be upper and lower gastrointestinal investigations, and thus a referral to gastroenterology would be warranted. It is important to rule out blood loss, in particular, through gastrointestinal investigations, before implicating poor dietary intake as the cause of the patient’s low iron stores and microcytic anemia.

The patient in question is at a high risk of sickle cell disease due to their ethnicity and family history. They are showing signs of parvovirus B19 infection, which is causing bone marrow failure and a decrease in erythropoiesis. This condition, known as aplastic crisis, is usually managed conservatively but may require a blood transfusion if the patient is experiencing symptomatic anemia. Granulocyte colony-stimulating factor (G-CSF) is not recommended in this case as it will not address the patient’s severe anemia. IV ceftriaxone and a lumbar puncture would be the correct initial management for meningococcal disease, but it is not the most likely diagnosis in this case. Oral benzylpenicillin and transfer to a pediatric ward is also not recommended as it is not the correct management for meningococcal disease and is not relevant to the patient’s condition. While sepsis is a possible differential diagnosis, the most likely cause of the patient’s symptoms is a viral infection causing aplastic crisis in a patient with sickle cell disease. Therefore, the appropriate management would be to investigate for viral infection and provide supportive therapies.

The diagnosis in this scenario is von Willebrand’s disease, which is the most common hereditary bleeding disorder caused by a defective von Willebrand factor. This protein plays a crucial role in haemostasis by assisting in platelet adhesion and stabilising coagulation factor VIII. A deficiency in von Willebrand factor prolongs bleeding time and APTT, but does not affect platelet counts or PT. It is more pronounced in women and may present with menorrhagia. Treatment involves administration of recombinant von Willebrand factor. Haemophilia A, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia, and vitamin K deficiency are other bleeding disorders with different causes and blood test results.

Different types of lipoproteins carry lipids and cholesterol throughout the body. Chylomicrons transport dietary lipids, VLDLs transport liver-synthesized lipids, LDLs carry cholesterol, and HDLs transport cholesterol back to the liver for breakdown. Fatty acids are broken down by pancreatic lipase and absorbed as free fatty acids and monoglycerides, which are then reformed into triglycerides and packaged into chylomicrons. The liver processes chylomicron remnants and liposomes into various lipoprotein forms, including VLDL and LDL. Apolipoproteins are proteins that bind to lipids to form lipoproteins. HDL particles remove cholesterol from circulation and transport it back to the liver. Oxidized LDL is harmful to the body and promotes atherosclerosis.

Causes of Macrocytic and Microcytic Anaemia

Anaemia is a condition characterized by a decrease in the number of red blood cells or haemoglobin in the blood. Macrocytic anaemia is a type of anaemia where the red blood cells are larger than normal, while microcytic anaemia is a type where the red blood cells are smaller than normal. Here are some of the causes of macrocytic and microcytic anaemia:

Alcohol Excess: Alcohol toxicity can directly affect the bone marrow, leading to macrocytic anaemia. Additionally, alcoholism can cause poor nutrition and vitamin B12 deficiency, which can also lead to macrocytosis.

Congenital Sideroblastic Anaemia: This is a rare genetic disorder that produces ringed sideroblasts instead of normal erythrocytes, leading to microcytic anaemia.

Iron Deficiency: Iron deficiency is a common cause of anaemia, especially in women. However, it causes microcytic anaemia, not macrocytic anaemia.

Blood Loss from Menses: Chronic blood loss due to menorrhagia can result in microcytic iron deficiency anaemia. However, this is a physiological process and would not cause macrocytic anaemia.

Thalassemia: Thalassaemia is a genetic disorder that leads to abnormal or low haemoglobin, resulting in microcytic anaemia.

Common Medications and Their Uses

Thrombocytosis and Hydroxyurea
Thrombocytosis is a condition characterized by an elevated platelet count, which can lead to bleeding or thrombosis. Primary or essential thrombocytosis is a myeloproliferative disorder that results in overproduction of platelets by the bone marrow. Hydroxyurea is the first-line treatment for essential thrombocytosis, as it inhibits an enzyme involved in DNA synthesis and reduces the rate of platelet production.

Interferon Gamma for Immunomodulation
Interferon gamma is an immunomodulatory medication used to reduce the frequency of infections in patients with chronic granulomatous disease and severe malignant osteopetrosis. It is administered by subcutaneous injection.

Cromoglycate for Inflammation
Sodium cromoglycate is a synthetic non-steroidal anti-inflammatory drug used in the treatment of asthma, allergic rhinitis, and various food allergies.

Interferon β for Multiple Sclerosis
Interferon β is a cytokine used in the treatment of relapsing-remitting multiple sclerosis. It is administered subcutaneously.

Ranitidine for Acid Reduction
Ranitidine is a H2 (histamine) receptor blocker that inhibits the production of acid in the stomach. It can be used in the treatment of gastro-oesophageal reflux disease, peptic ulcer disease, and gastritis.

Managing DIC in a Patient with Septic Shock: Evaluating Treatment Options

When managing a patient with disseminated intravascular coagulation (DIC), it is important to consider the underlying condition causing the DIC. In the case of a patient with septic shock secondary to a urinary tract infection, the sepsis 6 protocol should be initiated alongside pre-emptive management for potential blood loss.

While a blood cross-match is sensible, emergency blood products such as platelets are unwarranted in the absence of acute bleeding. Activated protein C, previously recommended for DIC management, has been removed from guidelines due to increased bleeding risk without overall mortality benefit.

Anticoagulation with low molecular weight heparin is unnecessary at this time, especially when given with blood products, which are pro-coagulant. Tranexamic acid and platelet transfusions are only warranted in the presence of severe active bleeding.

Prophylactic dose unfractionated heparin may be a good management strategy in the presence of both thrombotic complications and increased bleeding risk, but should be given at a treatment dose if deemed necessary. Ultimately, managing the underlying septic shock is the best way to manage DIC in this patient.