What is physiologic anemia of pregnancy
As the fetus develops, the erythropoietin levels rise, with the highest levels occurring in the final trimester. Erythropoiesis is directly driven by erythropoietin, and as a consequence, a significant portion of the red blood cell mass is produced in the final trimester of pregnancy. Upon birth, blood oxygen levels suddenly increase with the onset of breathing, closure of the ductus arteriosus, and transition of the newborn from the relatively hypoxic environment of the amniotic sac to oxygen-rich room air. Renal oxygen tension sensors detect this sudden rise in oxygen levels, and in response, downregulate hypoxia-inducible factors, which in turn, downregulate the production of erythropoietin. This results in a slowly decreasing hemoglobin for several weeks after birth, known as the physiologic nadir of infancy. In full-term infants, the hemoglobin typically reaches a nadir of approximately 11 g/dL (110 g/L) at 8 to 12 weeks after birth.

What is glanzman thrombasthenia?
The clinical findings seen in the girl in the vignette suggest a congenital bleeding disorder. Of note, she has had unusual bleeding since birth, repeated mucosal bleeding (such as gum bleeding while tooth brushing), palpable bruising, and petechiae. The normal prothrombin time (PT), partial thromboplastin time (PTT), and platelet number effectively rule out disorders of the coagulation cascade or platelet number. It is therefore likely that this girl has a disorder of platelet function. In platelet function disorders, platelets are present, but cannot activate to effectively form a clot. The most common congenital platelet function disorders are Bernard-Soulier syndrome (a disorder of platelet adhesion) and Glanzmann thrombasthenia (a disorder of platelet aggregation). The most appropriate management for life-threatening bleeding in a child with a known or suspected platelet function disorder is to transfuse functional platelets

What is Nephroblastoma?
Beckwith-Wiedmann's syndrome is a genetic syndrome due to del 11p15 characterized by:
Macrosomia (traditionally defined as height and weight >97th percentile)
Hemihyperplasia (asymmetric overgrowth of one or more regions of the body due to an abnormality of cell proliferation, [ie, increased cell number])
Macroglossia
Omphalocele/umbilical hernia/diastasis recti (figure 2 and picture 3)
Anterior linear ear lobe creases/posterior helical ear pits
Visceromegaly involving one or more intra-abdominal organs including liver, spleen, kidneys, adrenal glands, and pancreas
Embryonal tumors (eg, Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma) in childhood
Hypoglycemia

HOW DO YOU TREAT SICKLE CELL CRISIS?

WHAT IS THE MOST COMMON TRANSFUSION REACTION?

What is packed red cell transfusion?
The girl in the vignette has splenic sequestration. It is critical to rapidly recognize this event, as it can quickly result in very severe anemia and death. This sickle cell–related crisis occurs when sickling in the vasculature of the spleen entraps red blood cells, resulting in rapid splenic engorgement and a severe, potentially life-threatening anemia. Splenic sequestration is most common in children younger than 5 years, but can occur at any age. It typically presents, as in the child in the vignette, with signs of severe anemia (tachycardia, pallor, and fussiness), thrombocytopenia, and a palpable spleen. Fever may be present. One of the most effective interventions for reducing mortality in young children with sickle cell disease has been teaching parents to palpate for a spleen in their child daily. Splenic sequestration associated with sickle cell disease should be treated with a transfusion of packed red blood cells. If the anemia is very severe, the transfusion should be given slowly, over several hours, and typically in aliquots of 5 to 7 mL/kg. This approach avoids further cardiac stress. In addition, the spleen will eventually “release” the entrapped red blood cells, leading to a rapid rise in hemoglobin; if too much blood was transfused when this occurs, the child can experience a hyperviscous state.

What is tumor lysis syndrome?
Tumor lysis syndrome (TLS) is an oncologic emergency that is caused by massive tumor cell lysis with the release of large amounts of potassium, phosphate, and nucleic acids into the systemic circulation. Catabolism of the nucleic acids to uric acid leads to hyperuricemia, and the marked increase in uric acid excretion can result in the precipitation of uric acid in the renal tubules and can also induce renal vasoconstriction, impaired autoregulation, decreased renal blood flow, and inflammation, resulting in acute kidney injury. Hyperphosphatemia with calcium phosphate deposition in the renal tubules can also cause acute kidney injury.
Think PKU C
Phosphate high
Pottasium high
Uric acid high

C Calcium low
Prevented and treated with hyperhydration, allopurinol and alkaliinazation

HOW DOES SICKLE CELL ANEMIA AFFECT BLOOD CELLS?

WHAT IS THE FIRST STEP IN TREATING A TRANSFUSION REACTION?

What is Vit B12 deficiency anemia?
The differential diagnosis of macrocytic anemia in children includes vitamin B12 deficiency, folate deficiency, and bone marrow failure. Folate deficiency is most often dietary, typically in children whose diets rely heavily on goat milk. When vitamin B12 (also called cobalamin) is consumed, it attaches to haptocorrin and travels to the duodenum, where it is hydrolyzed and released from the haptocorrin. The free vitamin B12 then binds to gastric intrinsic factor and travels to the ileum. It is then absorbed in the ileum, enters the blood stream, and binds to transcobalamin. Vitamin B12 deficiency in pediatrics most often occurs either because of an absence of the terminal ileum (where B12 is absorbed), or because of a deficiency of intrinsic factor. The deficiency of intrinsic factor and subsequent vitamin B12 deficiency is called pernicious anemia. Congenital pernicious anemia occurs when there is a genetic defect resulting in hypofunctional or absent intrinsic factor. Pernicious anemia in adolescents typically results from gastric atrophy and achlorhydria caused by antibodies to the parietal cell and intrinsic factor. Left untreated, vitamin B12 deficiency results in a macrocytic anemia, in addition to neurologic symptoms including paresthesias, ataxia, and gait abnormalities, as a result of posterior and lateral spinal column degeneration. Vitamin B12 deficiency can be treated with the parenteral administration of vitamin B12.
The patient in the vignette has a medical history remarkable for autoimmunity, suggesting that her macrocytic anemia also has an autoimmune origin. Thus, it is likely that her macrocytic anemia is caused by autoimmune damage to the gastric parietal cells.

What is Burkitt's lymphoma
Burkitt lymphoma (BL) encompasses a group of highly aggressive B cell neoplasms with three distinct clinical forms: endemic, sporadic, and immunodeficiency-associated. Known translocations include responsible for this tumor include:
The Ig heavy chain gene on chromosome 14 – Resulting in the t(8;14)(q24;q32) found in 80 percent of BL.
The kappa light chain gene on chromosome 2 – Resulting in the t(2;8)(p11;q24) found in 15 percent of BL.
The lambda light chain gene on chromosome 22 – Resulting in the t(8;22)(q24;q11) found in 5 percent of BL

WHAT IS IMPORTANT BASIC PRINCIPLE TO TEACH SICKLE CELL PATIENTS?

WHAT IS THE ONLY IV SOLUTION COMPATIBLE WITH PRBC'S?

What is iron overload?
The size of red blood cells (RBC), as measured by the mean corpuscular volume (MCV), is in large part determined by the content of hemoglobin within the RBC. Any deficiency in the components of hemoglobin will, therefore, result in a low MCV. The 2 primary components of hemoglobin that can be deficient are iron or the globin protein. Hemoglobin A, the normal adult variant, consists of 2 β-globin chains and 2 α-globin chains, with the β-globin gene located on chromosome 11 and the α-globin gene on chromosome 16. Mutations resulting in reduced production of either α-globin or β-globin result in various thalassemia phenotypes and present with a microcytic anemia. The child in the vignette has a severe microcytic anemia and his hemoglobin electrophoresis pattern shows the absence of hemoglobin A (α2 β2), with only hemoglobin A2 (α2 δ2) and F (α2 γ2) present. This means that he has 2 dysfunctional β-globin genes, and therefore has β-thalassemia major. The most appropriate management of thalassemia major is chronic blood transfusions, typically every 3 to 4 weeks, to maintain a hemoglobin greater than 10 g/dL (100 g/L). With each transfusion of packed red blood cells (PRBC) comes a large iron load (each mL of PRBC delivers 0.75 mg of iron). The human body has no mechanism for eliminating excess iron, so iron accumulates with each transfusion. Patients with thalassemia who are treated with frequent transfusions are therefore at high risk for complications associated with iron overload.
The complications associated with chronic iron overload include endocrinopathies such as hypothyroidism, diabetes, hypogonadism, cardiomyopathy, and liver failure. Iron overload can be managed or even prevented through the use of aggressive chelation therapy. The most commonly used chelator is deferasirox, an oral, once a day medication. Some patients are unable to be adequately chelated with deferasirox and require the subcutaneous or intravenous administration of deferoxamine to maintain iron balance. The gold standard for assessing iron overload is liver biopsy, although newer techniques using specially calibrated magnetic resonance imaging are increasingly used.

WHAT IS THE MAJOR CAUSE OF DEATH IN LEUKEMIA PATIENTS?

WHAT BLOOD TYPE IS UNIVERSAL DONOR?

What is leucoreduced blood product?
The infusion of even small numbers of granulocytes can lead to the release of pro-inflammatory cytokines, thereby increasing the risk of a febrile transfusion reaction. In order to reduce this risk, granulocytes are removed from blood products at the time of initial processing or immediately prior to transfusion via filtration, a process called leukodepletion. For the girl in the vignette, leukodepletion of the red blood cells (RBC) prior to transfusion would have had the greatest likelihood of reducing her risk of fever.
Diphenhydramine reduces the risk of an allergic transfusion reaction through the blockade of histamine, but will not influence the risk of fever. While bacterial infections can certainly be transmitted through blood product transfusions and can lead to fevers, culturing the packed cells prior to transfusion is neither practical nor feasible. Once thawed, blood products need to be transfused prior to culture results being available.
Although granulocytes are removed through filtration, many lymphocytes are the size of RBC and are therefore not as effectively removed by filtration. While RBCs are typed by ABO typing, they are not human leukocyte antigen typed or matched to the recipient, and lymphocytes infused with a transfused product may recognize the recipient as foreign. In immune compromised hosts, the host immune system is unable to clear the infused donor lymphocytes, which may then undergo expansion in response to recognition of a foreign antigen and cause a severe, typically fatal, graft-versus-host reaction. In immune competent hosts, this does not occur, as the host immune system recognizes the donor lymphocytes as foreign and successfully eliminates them. Irradiation of blood products prior to transfusion renders donor lymphocytes replication incompetent, eliminating the threat of transfusion-associated graft-versus-host disease

WHAT TYPES OF BLOOD LOSS INDICATE A BLEEDING D/O ?

WHY ARE MOST HEMOPHILIACS MALE?

WHY USE A LARGE NEEDLE TO ADMINISTER BLOOD?