1. Blood and Blood-Forming Organs Diseases and Disorders
Chapter 7
Blood and Blood-Forming Organs Diseases and Disorders 1

2. Anatomy and Physiology
Hematologic system
Major functions of blood:
Transport nutrients to cells
Aid removal of wastes
Average adult has 5 to 6 liters of circulating blood 2

3. Blood Components 3

4. Anatomy and Physiology
Erythrocytes
Normal count:
4.2 to 6.3 million
Life span:
120 days
Form in bone marrow and do not reproduce 4

5. Anatomy and Physiology
Hemoglobin important in oxygen transport
Normal hemoglobin:
Male
13.5 to 18 hemoglobin in grams (g)/100 milliliters (ml)
Female
12 to 16 g/100 ml 5

6. Anatomy and Physiology
Leukocytes protect individual from infection
Normal count:
4,500 to 11,000 mm3
Platelets
Also known as thrombocytes
Important in blood clotting
150,000 to 350,000 mm3
Note: normal values may vary some from different laboratories or sources 6

7. Hematology
White Blood Cell
WBC
Non-Blacks: ,000/ml (4-10K/mL)
Blacks ,000/mL ( K/ul)
Hemoglobin
Hgb
M: g/dL
F: g/dL
Glysolated hemoglobin
HgbA1c
% of total Hgb
Hematocrit
Hct
M: 41-53%
F: 36-46%
Red blood cell
RBC
M: x 106 cells/mL
F: x 106 cells/mL
Mean corpuscular volume
MCV fL
Mean corpuscular hemoglobin
MCH
26-34 pg/cell
Mean corpuscular hemoglobin concentration
MCHC
gm/dL
Reticulocytes
Retic
x 103 cells/mL
Platelets
Plt
x 103/mL
Erythrocyte sedimentation rate
ESR
0-20 mm/hr

8. Anatomy and Physiology
Blood-forming organs:
Lymph nodes
Bone marrow
Spleen
Liver—the embryo makes blood cells at 6 weeks
Lymph system protects against pathogens 8

9. Anatomy and Physiology
Bone marrow
Major blood cell producer
Spleen produces lymphocytes, plasma cells, and antibodies
Filters microorganisms from blood
Liver produces prothrombin and fibrinogen for blood clotting 9

10. vitamin K and Liver Disease The liver stores vitamins and minerals to be used as needed including all the fat-soluble vitamins;(A, B12, D, E and K). .Vitamin K is a fat-soluble organic compound that the body needs to remain healthy. Although bacteria in the human intestine make some vitamin K, it is not nearly enough to meet the body's needs, so people must get most of their vitamin K from foods in their diet. Hepatitis C Cirrhosis; Vitamin K Deficiencies .The liver needs vitamin K to make factors that regulate blood clotting .Vitamin K deficiency is extremely rare in healthy people. It can, however, occur in individuals who have significant liver damage
or disease disorders
that interfere with the absorption of nutrients from the intestine. .

11. Liver disease (cirrhosis) causes a decrease in bile salt synthesis, leading to impaired absorption and Vitamin K deficiency. The liver
makes a number of coagulation (clotting) factors including prothrombin and fibrinogen. Most of these require the presence of vitamin
K to function normally. Vitamin K, as a fat-soluble vitamin, in turn requires bile salts to be absorbed properly by the body. If the liver
is impaired and does not produce enough bile this will have an effect on the capacity to clot blood; increasing the risk of bleeding.

12. 1-Vitamin K is necessary for the formation of prothrombin and other blood-clotting factors in the liver, and it also plays
a role in bone metabolism. ,2-A form of the vitamin is produced by bacteria in the colon and can be utilized to some degree.

13. Common Signs and Symptoms
Erythrocytopenia
Decrease in RBCs leading to anemia
Symptoms:
Fatigue
Headache
Low RBCs
Pallor
Shortness of breath 13

14. Common Signs and Symptoms
Erythrocytosis
Increased RBCs
Symptoms:
High RBCs
Reddened skin tones
Bloodshot eyes
Increased blood volume and pressure
Increased blood volume of heart 14

15. Common Signs and Symptoms
Leukocytopenia
Decreased white blood cells
Weakens immune system
Leukocytosis
Increased white blood cells
Normal response to acute infections 15

16. Common Signs and Symptoms
Thrombocytopenia
Decreased platelet count leading to coagulation problem
Symptoms:
Petechiae
Small hemorrhages in skin
Ecchymoses
Large areas of bruising or hemorrhage
Epistaxis, also called a nosebleed
Bleeding in mouth, gums, and mucous membranes 16

17. Common Signs and Symptoms
Thrombocytosis
Increase in platelets
Uncommon
Usually no serious side effects 17

18. Diagnostic Tests Complete blood count with differential and indices
Biopsy of blood-forming organs
Hematocrit (Hct) reflects amount of red cell mass as proportion of whole blood
Hemoglobin (Hgb) reflects blood’s oxygen-carrying potential 18

19. Complete Blood Count

20. Diagnostic Tests Bleeding time determines platelet disorders
E.g., hemophilia, thrombocytopenia, disseminated intravascular coagulation
Prothrombin time (PT), partial thromboplastin time (PTT), and international normalized ratio (INR) measure blood’s ability to clot 20

21. Diagnostic Tests
Bone marrow donation and transplant
Blood Tests 21

22. Bone marrow aspirations
Bone marrow biopsy
                                                                                                                                                      
Bone marrow aspirations

23. Coagulation Parameters
Prothrombin time PT
11-14 seconds
Partial thromboplastin time
PTT
25-35 seconds
International normalized ratio
INR
0.8 to 1.2

24. Disorders of Red Blood Cells
Anemia
Decrease in oxygen-carrying ability of RBC
Symptoms:
Pallor
Fatigue
Shortness of breath
Tachycardia
Headache
Irritability
Syncope 24

26. Disorders of Red Blood Cells
Iron deficiency anemia
Loss of iron or inadequate intake of iron
Causes:
Blood loss
Low dietary intake
Treatment:
Increase dietary intake of iron 26

27. Disorders of Red Blood Cells
Folic acid deficiency anemia
Folic acid needed for maturation of RBCs
Causes:
Poor diet
Overcooking of vegetables
Overconsumption of alcohol
Treatment:
Increase folic acid intake by eating green and yellow vegetables 27

28. Disorders of Red Blood Cells
Pernicious anemia
Lack of intrinsic factor leading to inadequate absorption of vitamin B12
Treatment:
Monthly injections of vitamin B12 for life 28

29. Uses
Cyanocobalamin is a man-made form of vitamin B12 used to treat low levels (deficiency) of this vitamin. Vitamin B12 helps your body use fat and carbohydrates for energy and make new protein. It is also important for normal blood, cells, and nerves. Most people get enough vitamin B12 in their diet, but a deficiency may occur in certain health conditions (e.g., poor nutrition, stomach/intestinal problems, infection, cancer). Serious vitamin B12 deficiency may result in anemia, stomach problems, and nerve damage.
How to use Vitamin B-12 injection
Before using, check this product visually for particles or discoloration. If either is present, do not use the liquid.

30. Benefits of Vitamin B Injections:
                                                 
*BoostEnergy *Increases activity of nervous system *Essential for acceleration of cell renewal *Enhances red blood cell production *Assists in stress management *Improves concentration and memory *Increases metabolism to burn fat *Removes and transports fat out of the body

31. Disorders of Red Blood Cells
Hemolytic anemia
Destruction of RBCs related to antibody–antigen reaction
Disorder of immune system leading to destruction of erythrocytes
Treatment:
Exchange transfusion and/or splenectomy 31

32. Severe Anemia
Infants are severely anemic with hemoglobins as low as 5 g/dl. This degree of anemia results in severe congestive heart failure that results in massive edema. The photo shows an infant with hydrops fetalis. Hydrops fetalis literally means bloated with fluid. The hepatosplenomegaly is also evident here. Infants will frequently have effusions in the pleural and pericardial cavities.

33. Disorders of Red Blood Cells
Sickle cell anemia
Hereditary
Found in African American race
Abnormal sickle shape of erythrocyte
Does not allow cell to travel smoothly through vessels
Symptomatic treatment
No cure
The next slides explain and show some complications from sickle cell 33

34.  Sickle-cell disease is the most prevalent genetic disease in the Brazilian population. Lower limb ulcers are the most frequent cutaneous complications, affecting 8% to 10% of the patients. These ulcers are usually deep and may take many years to heal. Evidence about the effectiveness of systemic or topical treatment of these wounds is limited, apart from stabilization of the anemia - See more at:

35. Aplastic Crises In Sickle Cell Anemia

37. Disorders of Red Blood Cells
Hemorrhagic anemia
Loss of whole blood
Also called blood loss anemia
Complication is hypovolemic shock
Symptoms include:
Dizziness, fainting, thirst
Treatment
Stop bleeding
Oxygen
Restore blood volume 37

38. Disorders of Red Blood Cells
Aplastic anemia
Failure of bone marrow to produce blood components
Causes:
Chemotherapy
Radiation
Viruses
Toxins
Treatment:
Avoid causative agent
Bone marrow transplantation
Transfusions 38

39. Disorders of Red Blood Cells
Polycythemia
Too many blood cells
Symptoms:
Enlarged spleen
Reddened mucous membranes
Bloodshot eyes
Deep red color on palms
Treatment:
Donate blood at regular intervals to reduce blood volume 39

41. Polycythemia

42. Disorders of White Blood Cells
Mononucleosis
Caused by virus
Symptoms:
Fatigue
Sore throat
Swollen glands 42

44. Disorders of White Blood Cells
Leukemia
Malignant neoplasm of blood-forming organs
Abnormal production of immature leukocytes
May be acute or chronic
Acute forms affect children, progress rapidly, and may be fatal ---Most childhood leukemias are acute lymphocytic leukemia (ALL). Most of the remaining cases are acute myeloid leukemia (AML). 44

45. About Leukemia
Leukemia is a type of cancer that affects the body's white blood cells (WBCs).
Normally, WBCs help fight infection and protect the body against disease. But in leukemia, WBCs turn cancerous and multiply when they shouldn't, resulting in too many abnormal WBCs, which then interfere with organ function.
If too many lymphoblasts (a certain type of WBC) are produced, a child will develop acute lymphoblastic, or lymphoid, leukemia (ALL). This is the most common type of childhood leukemia, affecting about 75% of kids with this cancer of the blood cells. Kids ages 2 to 8 are more likely to be affected, but all age groups can develop ALL.

46. Children who have inherited certain genetic problems (such as Li-Fraumeni (The syndrome is linked to germline mutations of the TP53 tumor suppressor gene) syndrome, Down syndrome, Klinefelter syndrome, neurofibromatosis, (Neurofibromatosis is a genetic disorder that disturbs cell growth in your nervous system, causing tumors to form on nerve tissue) ataxia telangiectasia, or Fanconi anemia (The Fanconi anemia (FA) pathway is one of the DNA damage response mechanisms frequently lost in cancer. also have a higher risk of developing leukemia, as do those who are receiving medicines to suppress their immune systems after organ transplants.
Kids who have received radiation or chemotherapy for other types of cancer also have a higher risk, usually within the first 8 years after treatment.

47. Disorders of White Blood Cells
Leukemia
Chronic forms affect older adults, are often asymptomatic, and may not be fatal
Classified as:
Myelogenous
Affecting bone marrow
Lymphocytic
Affecting lymph nodes 47

49. Disorders of White Blood Cells
Leukemia
Symptoms:
Fatigue
Headache
Sore throat
Dyspnea
Bleeding of mucous membranes of mouth and gastrointestinal (GI) system
Bone and joint pain
Enlargement of lymph nodes, liver, and spleen
Infections common 49

50. Disorders of White Blood Cells
Leukemia
Diagnosis by bone marrow biopsy
Treatment:
Aggressive chemotherapy
Once in remission, bone marrow transplant to replace neoplastic tissue with normal tissue
Remission at 50% 50

51. Bone marrow biopsy

52. Disorders of White Blood Cells
Hodgkin’s disease
Most common lymphoma
Symptoms:
Painless enlargement of lymph nodes in neck
Weight loss
Fever
Primarily affects young adults
Average age of 35 52

53. For Hodgkin and NHL, the symptoms are the same
For Hodgkin and NHL, the symptoms are the same. However, one key feature in Hodgkin Lymphoma is the Pel-Ebstein fever or cyclic fever, which increases and then suddenly decreases within 1 – 2 weeks, and starts all over again.
A very important symptom in all types of lymphomas is the painless enlargement of the lymph nodes (in the neck, mandible, axillary, shoulder, etc.).

54. Then, fever, chills, night sweats and weight loss might ensue
Then, fever, chills, night sweats and weight loss might ensue. As the disease progresses, the enlarged lymph nodes could become painful, leading to what is referred to as “painful lymphadenopathy”. Some patients could also experience intractable back pain that doesn’t seem to be relieved by any medications. Also, bleeding under the skin (ecchmymoses, purpurae or petechiae) could also be observed in patients suffering from lymphomas.

55. Disorders of White Blood Cells
Hodgkin’s disease
Cause thought to be viral
Diagnosis by presence of Reed–Sternberg cell in lymphatic tissue
Treatment:
Radiation
Chemotherapy
If in remission for 5 years or more, complete cure possible 55

56. Disorders of White Blood Cells
Non-Hodgkin’s lymphoma (NHL)
Lymphomas lacking Reed–Sternberg cell
Symptoms:
Painless enlargement of lymph nodes of neck, axilla, and inguinal areas
Fever
Night sweats
Weight loss 56

57. Other Clues
In Hodgkin Lymphoma, the affected lymph nodes are contiguous. In other words, they follow a specific chronological pattern” starting in the shoulders, then the neck, then the chest. Non-Hodgkin lymphomas however could arise anywhere and affect any type of lymph nodes at a given time, with no specific pattern.

58. Treatment for Non-Hodgkin’s
Treatment for lymphoma that causes signs and symptoms
If your non-Hodgkin's lymphoma is aggressive or causes signs and symptoms, your doctor may recommend treatment. Options may include:
Chemotherapy. Chemotherapy is drug treatment — given orally or by injection — that kills cancer cells. Chemotherapy drugs can be given alone, in combination with other chemotherapy drugs or combined with other treatments.
Radiation therapy. Radiation therapy uses high-powered energy beams, such as X-rays, to kill cancerous cells and shrink tumors. During radiation therapy, you're positioned on a table and a large machine directs radiation at precise points on your body. Radiation therapy can be used alone or in combination with other cancer treatments.

59. Cont.
Stem cell transplant. A stem cell transplant is a procedure that involves very high doses of chemotherapy or radiation with the goal of killing the lymphoma cells that may not be killed with standard doses. Later, healthy stem cells — your own or from a donor — are injected into your body, where they can form new healthy blood cells.
Medications that enhance your immune system's ability to fight cancer. Biological drugs help your body's immune system fight cancer. Rituximab (Rituxan) is a type of monoclonal antibody that attaches to B cells and makes them more visible to the immune system, which can then attack. Rituximab lowers the number of B cells, including your healthy B cells, but your body produces new healthy B cells to replace these. The cancerous B cells are less likely to recur.

60. Cont.
Medications that deliver radiation directly to cancer cells. Radioimmunotherapy drugs are made of monoclonal antibodies that carry radioactive isotopes. This allows the antibody to attach to cancer cells and deliver radiation directly to the cells. Two radioimmunotherapy drugs — ibritumomab (Zevalin) and tositumomab (Bexxar) — are used to treat lymphoma.

61. Disorders of White Blood Cells
Multiple myeloma
Malignant neoplasm of plasma cells or B-lymphocytes
Increases with age
Peaks in 70s
Plasma cells multiply abnormally in bone marrow 61

62. Disorders of White Blood Cells
Multiple myeloma
Diagnosis by honeycombed bone pattern, hypercalcemia, Bence Jones protein found in blood and urine, and bone marrow biopsy
Treatment:
Chemotherapy
Radiation
Not effective
Poor prognosis 62

63. myeloma
Multiple myeloma causes cancer cells to accumulate in the bone marrow, where they crowd out healthy blood cells. Rather than produce helpful antibodies, the cancer cells produce abnormal proteins that can cause kidney problems.
Treatment for multiple myeloma isn't always necessary. If you're not experiencing signs and symptoms, you may not require treatment. If signs and symptoms develop, a number of treatments can help control your multiple myeloma

64. Disorders of Platelets
Hemophilia
X-linked hereditary bleeding disorder
Several types
Type A
Most common
Male children from asymptomatic mothers
Lack protein necessary for clot formation 64

65. Disorders of Platelets
Hemophilia
Symptoms:
Epistaxis
Bruising
Prolonged bleeding
Diagnosis by blood test 65

66. Disorders of Platelets
Hemophilia
Treatment:
Prevention of injury
Treat symptoms
Blood transfusions
Concentrated form of clotting protein
No cure 66

67. Hemophilia usually is inherited
Hemophilia usually is inherited. "Inherited” means that the disorder is passed from parents to children through genes.
The two main types of hemophilia are A and B. If you have hemophilia A, you're missing or have low levels of clotting factor VIII (8). About 8 out of 10 people who have hemophilia have type A. If you have hemophilia B, you're missing or have low levels of clotting factor IX (9).

68. Rarely, hemophilia can be acquired
Rarely, hemophilia can be acquired. "Acquired” means you aren't born with the disorder, but you develop it during your lifetime. This can happen if your body forms antibodies (proteins) that attack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors from working.

69. Disorders of Platelets
Thrombocytopenia
Also known as thrombocytopenia purpura
Decrease in platelets leading to inability to normally clot blood
Symptoms:
Abnormal bleeding in skin, mucous membranes, and internal organs
Petechiae 69

71. Disorders of Platelets
Thrombocytopenia
Symptoms:
Ecchymoses
GI hemorrhages
Epistaxis
Hematuria 71

72. Disorders of Platelets
Thrombocytopenia
Diagnosis by platelet count and bleeding time
Treatment:
Avoid tissue trauma to reduce bleeding
Vitamin K
Transfusions of platelets
Splenectomy 72

73. Disorders of Platelets
Disseminated intravascular coagulation (DIC)
Abnormal clotting followed by abnormal bleeding
Usually follows major trauma
E.g., complicated childbirth, surgery, tissue destruction, septicemia, snakebite, shock
Multiple clots in capillaries
Life-threatening 73

74. Some diseases and conditions can cause clotting factors to become overactive, leading to DIC. These diseases and conditions include:
Sepsis (an infection in the bloodstream)
Surgery and trauma
Cancer
Serious complications of pregnancy and childbirth
Examples of less common causes of DIC are bites from poisonous snakes (such as rattlesnakes and other vipers), frostbite, and burns.

75. DIC can occur in the following conditions:
Solid tumors and hematologic malignancies (particularly acute promyelocytic leukemia)
Obstetric complications: abruptio placentae, pre-eclampsia or eclampsia, amniotic fluid embolism, retained intrauterine fetal demise, septic abortion
Massive tissue injury: severe trauma, burns, hyperthermia, rhabdomyolysis, extensive surgery
Sepsis or severe infection of any kind (virtually infections by any microorganism can cause DIC, though bacterial infections are the most common): bacterial (Gram-negative and Gram-positive sepsis), viral, fungal, or protozoan infections
Transfusion reactions (i.e., ABO incompatibility hemolytic reactions)
Severe allergic or toxic reactions (i.e. snake or viper venom)
Giant hemangiomas (Kasabach-Merritt syndrome)

76. Disorders of Platelets
DIC
Symptoms:
Petechiae
Ecchymosis
Hematoma
Hematuria
GI bleeding
Hematemesis
Blood in stool
Treatment:
Heparin or infusion of platelets 76

77. Rare Diseases Thalassemia Von Willebrand’s disease ( Lymphosarcoma
Primarily affects people of Mediterranean descent
Fragile, thin RBCs form defective hemoglobin
Von Willebrand’s disease (
Hereditary, congenital
Deficiency in clotting factor and platelet function
Lymphosarcoma
A type of lymphoma
Symptoms similar to Hodgkin’s disease 77

78. Thalassemias (thal-a-SE-me-ahs) are inherited blood disorders
Thalassemias (thal-a-SE-me-ahs) are inherited blood disorders. "Inherited" means that the disorder is passed from parents to children through genes. 
Thalassemias affect males and females. The disorders occur most often among people of Italian, Greek, Middle Eastern, Southern Asian, and African descent. Severe forms usually are diagnosed in early childhood and are lifelong conditions

79. Thalassemias (thal-a-SE-me-ahs) are inherited blood disorders
Thalassemias (thal-a-SE-me-ahs) are inherited blood disorders. "Inherited" means that the disorder is passed from parents to children through genes. 
Thalassemias cause the body to make fewer healthy red blood cells and less hemoglobin (HEE-muh-glow-bin) than normal. Hemoglobin is an iron-rich protein in red blood cells. It carries oxygen to all parts of the body. Hemoglobin also carries carbon dioxide (a waste gas) from the body to the lungs, where it's exhaled.
People who have thalassemias can have mild or severe anemia (uh-NEE-me-uh). Anemia is caused by a lower than normal number of red blood cells or not enough hemoglobin in the red blood cells.

80. Von Willebrand
Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.

81. [lim-foh-sahr-koh-muh]
lymphosarcoma
[lim-foh-sahr-koh-muh]
a malignant tumor in lymphatic tissue, caused by the growth
of abnormal lymphocytes.
Lymposarcoma

82. Effects of Aging
Age-related changes in other systems (e.g., immune, digestive) leave older adults more susceptible to infections and nutritionally related blood disorders
Decreased iron
Most common disorder is anemia
Poor nutrition 82