Week 8- Chronic Alterations in Hematology and Endocrinology

Iron deficiency anemia (IDA)

decreased hemoglobin and iron

IDA is caused by inadequate ____ and chronic ____

dietary intake of iron, blood loss

Common causes of IDA blood loss in females

pregnancy and menorrhagia

Common causes of IDA blood loss in males

ulcers, hiatial hernia, esophageal varacies, cirrhosis, hemorrhoids, ulcerative colitis, and cancer

IDA is a what type of anemia?

microcytic hypochromic (small cells, low hemoglobin)

Most common cause of IDA in children is

inadequate intake

IDA is the most common nutritional deficiency in

children

If IDA is untreated in children, it can cause irreversible effects on

development

Children are at risk for IDA due to _____ and ____

rapid growth, poor eating habits

Cows milk can cause an

increased risk of developing IDA

What aged children are most at risk for IDA?

6 months to 2 yrs

IDA in children can cause ____ deficiency

intelectual/behavioural

Chronic IDA 3 main causes

parasitic infection, hemorrhagic disease, or PUD

IDA is also associated with the

SEDOHs (poverty/developing countries)

4 main causes of IDA (diet and blood loss related) include

dietary deficiencies, impaired absorption, increased metabolic requirements, and chronic blood loss

Most common dietary deficiency that causes IDA is

inadequate protein intake

Other dietary deficiencies that cause IDA include

vegetarian diet and PICA

PICA

an abnormal craving for nonfood substances, such as dirt, paint, or clay

Impaired absorption causing IDA includes ______, ______, and _____

partial/total gastrectomy, chronic diarrhea, and malabsorption syndrome

Increased metabolic requirements causing IDA includes _____ and _____

pregnancy, lactation

Chronic blood loss causing IDA includes ______ and _____

GI bleed, menstrual loss

Common cause of GI bleeds are ____ and ____

ulcers, chronic NSAID use

A surgical procedure that could cause IDA (blood loss ) is a

gastric bypass

Impaired absorption causes of IDA in children

celiac disease, chronic diarrhea, and malabsorption syndrome

Increased requirement cause of IDA in children is due to

growth spurts

Other causes of IDA in children are ____, ____, and ____

GI lesions, parasitic infections, hemorrhagic disease

Stage 1 IDA

low iron, normal RBC, and normal hemoglobin

Stage 2 IDA

low iron, abnormal RBC production

Stage 3 IDA

hemoglobin-deficient RBC enter the circulation, clinical manifestations appear

Iron deficiency anemia early clinical manifestations

fatigue, weakness, shortness of breath, increased HR, and pale earlobes/palms/conjunctiva

IDA clinical manifestations (neuro)

irritability, decreased activity tolerance/weakness, headaches, and confusion/memory loss in older adults

IDA clinical manifestations (resp)

dyspnea

IDA clinical manifestations (GI)

PICA, smooth/sore/red/beefy tongue, glossitis, dysphagia, gastritis

Dysphagia in IGA commonly occurs due to

web of mucus and inflammatory cells forming at the base of the esophagus

IDA clinical manifestations (integ)

angular stomatitis and spoon shaped nails

Angular stomatitis

cracks in the corners of the mouth

Mild IDA clinical manifestations in children

lethargy, listlessness, irritability, decreased activity tolerance, weakness, and lack of interest in play (anhedonia)

Mild IDA hemoglobin levels

70-100 (can usually be treated with diet)

Severe IDA clinical manifestations in children

pallor, tachycardia, systolic murmurs, splenomegaly, widened scull sutures, developmental delays, decreased physical growth, PICA, cognitive impairment

Severe IDA hemoglobin levels

<70 (or symptomatic)

What is not an indicator of IDA in children?

weight

Hemoglobin levels for packed RBC transfusion usually occurs when hemoglobin drops <_____ or patient is _____

70, symptomatic

Diagnostic tests for IDA include

CBC, serum iron, red cell distribution width (RDW), and rarely bone marrow biopsy (other tests are also performed, all have to do with iron or cell count)

Primary hemochromatosis is a hereditary

autosomal recessive disorder

Primary hemochromatosis is characterized by increased

GU iron absorption and tissue iron deposition (too much iron)

Where is excess iron first deposited?

liver and pancreas

Following the liver and pancreas, where is the excess iron stored?

heart, joints, and endocrine glands

Secondary causes of hemochromatosis include

anemias with inefficient erythropoiesis (siderblastic anemia, aplastic anemia), dietary iron overload, or conditions that require repeated blood transfusions

Hemochromatosis iron accumulation in the body occurs due to deficiency of

hepcidin

Hepcidins job is to

decrease plasma iron levels

Iron overload in hemochromatosis results in

liver fibrosis, cirrhosis, hepatocellular carcinoma, diabetes, hypothyroidism, arthritis, cardiomyopathies, and skin hyperpigmentation

Major goal of hereditary hemochromatosis screening and treatment is to prevent

cirrhosis

Hemochromatosis clinical manifestations (neuro)

fatigue and malaise

Hemochromatosis clinical manifestations (CV)

cardiomegaly (usually restrictive), ascites, and low albumin

Hemochromatosis clinical manifestations (GI/GU)

abdominal pain, impotence, hepatomegaly, abnormal liver enzymes, and diabetes

Hemochromatosis clinical manifestations (Integ/MSK)

arthralgias and bronzed skin

Juvenile hemochromatosis usually presents before the age of

30

Juvenile hemochromatosis clinical manifestations (female)

fail to start menstrual cycle at proper age or absent and erratic periods/stops once it begins

Juvenile hemochromatosis clinical manifestations (male)

hypogonadism

Juvenile hemochromatosis clinical manifestations (other)

cardiomyopathy, a-fib, jaundice or odd colour skin (ashen grey/green/reddish), RUQ pain (liver), gallbladder/pancreas/liver problems, joint pain, rapid weight loss, irregular heartbeat, and elevated blood sugars

Due to liver involvement in hemochromatosis we should always monitor

LFTs

When assessing our patient for hemochromatosis we want to ask about their

diet

Diet to improve hemochromatosis includes

reduce consumption of red meat, avoid foods high in animal fat, limit vitamin C intake, decrease alcoholic beverages, avoid sugary foods/beverages, consume fruits/veggies, eat grains/nuts/rice/beans, avoid raw shellfish, and have meals with tea or coffee (reduces iron absorption)

Foods that contain ___ will decrease iron absorption

tannins

Sickle cell disorders are a hereditary condition where there is a replacement of normal hemoglobin with

abnormal hemoglobin

Chronic hemolytic anemia has no

cure (life long)

Sickle cell trait (most common)

1 normal hemoglobin gene and 1 sickle cell hemoglobin gene, child is a carrier of sickle cell anemia and rarely has symptoms (heterozygous)

Sickle cell anemia

2 sickle hemoglobin genes, child is subject to sickle cell crisis

Children under 5 with sickle cell disease are at increased risk for

infection, sepsis, and death

Most people with sickle cell anemia live into their

5th decade

During episodes of sickling, RBCs become ___ shaped

cresent

What three factors can increase the rate of sickling?

oxygenation, pH, and hydration (dehydration)

Decreased oxygenation causes

increased sickled cells

Lowered pH causes

increased sickled cells

Dehydration causes

increased sickled cells

Repeated sickling/unsickling weaken the RBC membranes and they are then

hemolyzed and removed

Sickle cells can regenerate (after persistent hypoxemia) when they are adequately

hydrated and oxygenated

Sickle cell disease decreases the _____ and increases the demand for ____

lifespan of RBCs, RBC production

When sickled cells occlude a blood vessel it can cause ___ and ____

ischemia, organ damage

Sickle cell diseases have an increased risk for ischemia/clots causing

strokes, PE, blockages of capillaries in kidneys, etc.

Sickle cell disease labs

CBC (hemoglobin/platelets/WBC) and creatinine/BUN

Individuals with sickle cell disease have severe ___ anemia

hemolytic (breakdown faster than replaced despite erythropoiesis)

Certain populations have an increased

risk for sickle cell diseases

Other precipitating factors for sickling include

low environmental/body temperature (altitude), excessive exercise, dehydration, infections, fever, and pregnancy

Sickle cell anemia diagnostics (newborns/children)

cord blood test (newborns), genetic testing, sickle turbidity test, and hemoglobin electrophoresis

Sickle turbidity test

tests hemoglobin in children >6 months once the fetal hemoglobin levels fall

Sickle cell anemia may be triggered by ____ and _____

fever, emotional stress

Sickle cell anemia can increase patient risk for

acute chest syndrome, stroke, infection, and priapism

Acute chest syndrome

sickle cells get trapped in the lungs

Acute chest syndrome symptoms include

fever, chest pain, progressive respiratory distress, increased WBC count, and pulmonary infiltrates

Priapism

persistent and painful erection

Sickle cell anemia HBSS acronym (clinical manifestations)

H- hemolysis

B- bone marrow hyperplasia/infarction

S- stroke

S- skin ulcers

Sickle cell anemia PAIN acronym (clinical manifestations)

P- pain/psychosocial problems/priapism

A- anemia/aplastic crisis/avascular necrosis

I- Infections

N- nocturia (frequent urination)

Sickle cell anemia CRISIS acronym (clinical manifestations)

C- chest syndrome/cardiomegaly/HF

R- retinopathy/renal failure

I- Infarction

S- sequestration crisis

I- increased fetal loss during pregnancy

S- sepsis

Sickle cell crisis occurs when the % of sickled hemoglobin ____ resulting in the appearance of symptoms usually marked by _____

increases, acute pain from ischemia

Acute ischemic pain usually occurs in

joints, heart, or kidneys

3 types of sickle cell crisis

vaso-occlusive, splenic sequestration, aplastic

Most common sickle cell crisis is

vaso-occlusive crisis (can occur over days/weeks)

Vaso-occlusive crisis occurs when stasis of blood causes sickle cells to _____, causing ____

clump in vasculature, ischemia/infarction

What may occur to local tissue if the vaso-occlusive crisis is not reversed?

thrombosis and infarction causing renal failure, AMI, strokes, etc.