heme and lymph patho

what is hemophilia

bleed too damn much

what is hematocrit

% of rbc’s

what is plasma made of

water, proteins, nutrients, hormones, waste, na, cl, k

what is anemia

too little hematocrit

what is polycythemia

hematocrit too high, blood may be too thick

what do rbc’s come from

reticulocytes

what do platelets come from

megakaryocytes

how do you make tissue macrophage

blast cells → monocytes → tissue macrophage

how do you make neutrophils

blast cells → promyelocyte → band cell → neutrophil

what do B and T lymphocytes come from

bone marrow lymphocyte precursors

*how long do rbcs stay in circulation

120 days

*what is A1C and why is it measured in diabetic patients every 3-4 months

its the amount of sugar carried on 1 hemoglobin, which lives for 120 days. allows providers to see a “average blood sugar” instead of just the patients current blood sugar

*what does MCV measure

how big each rbc is

*what does low MCV mean

anemia caused by chronic kidney disease

*what does MHC measure

average amount of hemoglobin in rbcs

*what does low mhc mean

hypochromic anemia

*what does high mhc mean

dehydration, hemolytic anemia

what is a retic count measuring

% of immature rbcs in blood

what does a high retic count mean

bone marrow is making more rbc’s and trying to fix anemia

what makes up rbcs

2 alpha chains, 2 beta chains, 4 heme groups that hold iron

*what are high neutrophil levels associated with

BACTERIAL INFECTIONS

*what are high lymphocyte levels associated w

viral infections

*what are high monocyte levels associated with

fungal infections, TB

*what are high eosinophil levels associated with

allergic reactions, autoimmune disorders, parasites

*what are high basophils levels associated with

cancer

*what is low platelet count associated with

chronic liver disease

*what is dec production of rbcs associated with

bone marrow failure (APLASTIC ANEMIA)

what nutrient deficiencies may lead to anemia

iron, B12, folate, protein

what do low levels of B12 or folate mean

megaloblastic anemia

what is the most common nutrient deficiency in the world

iron (microblastic hypochronic)

what does protein deficient anemia entail

decreased formation of all homopoetic cells, liver cirrhosis bc liver cant make proteins and thus all cells are protein deficient

what are examples of genetic hemoglobinopathies that lead to weird hemoglobin and rbc shapes

sickle cell anemia, thalassemia

what is an example of structural preotein defects that lead to abnormal rbc shapes

spherocytosis

causes of increased loss or death of rbcs

bleeding, immune hemolytic anemia, hypersplenism, malaria

microcytic hypochronic anemia =

iron deficient anemia

macrocytic normochromic anemia=

b12 deficient (vegans, gastric bypasses)

*what does dec wbc and platelets mean, how is it treated

pancytopenia, need transplanted stem cells

causes of aplastic anemia

idiopathy (most common) or secondary bc of chemo, radiation or a viral infection

clinical features and treatment for aplastic anemia

recurrent infections bc of pancytopenia and bleeding, need a bone marrow transplant

clinical features and treatment for iron deficient anemia

pale, tired, dyspnea. stop the bleeding and get em some iron

causes of iron deficient anemia

inc loss of iron, dec intake or absorption of iron, inc iron needs

what would inc loss or iron

chronic bleeding, menorrhagia, bleeding ulcer, use too many NSAIDS, hookworms

what would cause dec iron intake/absorption

malnutrition (intake), chrons, celiac, inc use of antiacids

what would cause inc iron requirement

being preg, growing kids/teens, period

where is 60-80% of bodys iron

in rbcs

what is iron doing as its metabolized

most bound to ferratin or transferatin going to live/bone marrow

what binds to b12 after its separated from protein by acid during its metabolism

intrinsic factor

*causes of megaloblastic anemia

pernicious- atrophic gastritis and lack of intrinsic factor antibodies prevent binding of b12 to the intrinsic factor, gastric bypass, celiac, chrons

what do pernicious megaloblasic anemai look llike in bone marrow

bone marrow is hypercellular and has lots of megaloblasts, decreased but huge rbcs

what happens if you dont treat pernicious megaloblastic anemia

spinal cord disease, demyelination and neuro symptoms if severe

clinical features and treatment of megaloblastic anemia

tired, pale, sob, weak, hard to walk and look watch their own legs when they walk. loss of proprioception, deep tendon reflexes and sense of vibration. spinal cord issues if severe. need folate or b12 supplements

causes of hemolytic anemias

structural abnormalities or infection, leading to rbc destruction

common features of hemolytic anemias

reduced rbc lifespan, inc erythropoietin, erythroid hyperplasia*, byperbilirubenemia, jaundice

what causes sickle cell anemia

genetic, an abnormal hemoglobin S gene in some or all rbcs

% of rbcs w hemoglobin S mutation and associated symptoms

less than 40%- asymptomatic

40-80%- mild symptoms

80%+- severe symptoms

what can lead to sickling crisis’s (flare ups of sickle cell anemia)

sickness, low o2 pressure, hard workouts, pregnancy

clinical features of sickle cell anemia

dec/slow intellectual development and neurologic deficits, cardiopulmonary insuffciency, recurrent infections, anemia, elevated bilirubin

*cause of thalassemia

genetic defect in synthesis of HbA so less globin is made

which kind of thalassemia is worst (and more common) and why

T beta, bc only two beta chains so harder to compensate if one is fucked, but you have 4 alpha chains so if one or two are fucked its easier to compensate

clinical features of thalassemia

microcytic, hypochromic. slow growth, T major specifically- hepatosplenomegaly hemosiderosis (iron overload)

treatment for thalassemia

no cure, T minor- no treatment needed, T major- kids will die from lack of hemoglobin unless transfused

causes of hereditary spherocytosis

genetic defect of structural proteins of rbcs, causes membrane destabilization and lysis in spleen, big ass round spherocytes cant get into tiny capillaries

clinical features of hereditary spherocytosis

typical anemia shit, splenomegaly and jaundice from the hemolysis

treatment for hereditary spherocytosis

splenectomy (doesnt fix the spherocytes not being able to go into capillaries tho)

causes of immune hemolytic anemia

IgG binds to rbc autoantigen/neoantigen→ activates compliment→ rbc lysis

clinical features of immune hemolytic anemia

splenomegaly, jaundice, rapid drop of Hb

*causes of polycythemia

neoplastic (cancer of blood, most common), blood doping

clinical features of polycythemia

hyperviscosity, clots easy, hypertension bc too much blood in vessels, flushed, erythroid hyperplasia in bone marrow, bone marrow cells look weird

treatment for polycythemia

phlebotomy, chemo drugs

*neutrophils are _%

54-62%

*lymphocytes are _%

25-33%

*monocytes are _%

3-9%

*eosinophils are _%

1-3%

*basophils are _%

less than 1%

*hemolytic anemia intracorpuscular defects cause

structural abnormalities

*hemolytic anemia extracorpuscular defects cause

antibodies (transfusion rxn or autoantibodies), infection, mechanical factors

*causes of leukopenia

can be part of aplastic anemia (presents as pancytopenia), chemo, environment, radiation

clinical features of leukopenia

neutropenia → frequent bacterial infections

lymphopenia → frequent bacterial, viral, fungal and/or parasitic infections bc low lymphocyte amount

long term may be fatal

leukopenia treatment

stop exposure to whatever is causing it or give poietins to inc rbc prod

what does persistent leukocytosis look like and need

giant tender lymph node in neck of kid/teen that doesnt respond to antibiotics, may need a biopsy

wbc and plasma malignancies causes

idiopathic or caused by a virus

wbc and plasma malignancies clinical features

bone marrow infiltration by malignant cells, inc number of immature blood cells, anemia, recurrent infections, uncontrolled bleeding, neoplastic stem cells show genetic changes

*acute lymphocytic leukemia epidemiology

20% of all leukemias but most common leukemia in kids, rapid progression, peaks age 5, bad prognosis in elderly

*acute myoloid leukemia epidemiology

*auer rods on smear, most common acute leukemia in adults mostly elderly, 40% of all leukemias, myeloproliferative disease, w/o treatment 6mo survival, w treatment 15-30%

acute myoloid leukemia classification

AML w recurrent genetic abnormalities, AML evolving from multilineage dysplasia, AML not otherwise specified

acute myoloid leukemia treatment

chemo, high dose irradiation, bone marrow transplant

chronic myoloid leukemia epidemiology

15% of all leukemias, rare before adolescence, incidence inc w age , most pts die in 3 yrs, malignant disease of pluripotent hematopoietic stem cells (90% philadelphia better prognosis)(most promising is gleevec- 90%)

chronic myoloid leukemia clinical features

slow onset, mild anemia, hypermetabolism, tire easily, prone to infections, splenomegaly and clotting

chronic phase of chronic myoloid leukemia

2-3yrs, marked leukocytosis, bone marrow < 10 blasts, inc platelets in bone marrow

accelerated phase of chronic myoloid leukemia

(1/2 pts) over 10% blasts in bone marrow, over 20% basophils in blood, increasingly unresponsive to treatment, ends in blast crisis

sudden onset blast crisis phase of chronic myoloid leukemia

other (1/2 pts), cant be treated, dead

chronic lymphocytic leukemia epidemiology

25% of all leukemias, most over 50, slow progression, can transform into another leukemia with less favorable prognosis

chronic lymphocytic leukemia clinical features

smudge cells on smear, indistinguishable from normal lymphs, similar to small-cell lymphocytic lymphoma

chronic lymphocytic leukemia treatment

unresponsive to chemo

*lymphomas epidemiology

3% of all malignant disease, no benign ones, most lymphomas have a B-cell phenotype, all ages, often infiltrate the lymph nodes/spleen/thymus/bone marrow, diagnosed by lymph node biopsy w light microscopy

non hodgkins lymphoma most often involves what

lymph nodes, bone marrow, spleen, and thymus but can be extranodal (GI)

clinical features of non hodkins lymphoma

lymph node enlargement (solo or diffuse), tired, fever, weight loss, sweating, anemia, leukopenia, infections

features of follicular non hodgkins lymphoma

most common in US, most common in elderly, slow growing, usually mild constitutional, chemo ineffective, in terminal stage body overwhelmed by tumor burden

features of diffuse large B cells non hodgkins lymphoma

several forms, most common aggressive NHL, tumor cells spread, chemo works

features of burkitts non hodgkins lymphoma

highly malignant, cells prone to apoptosis, chemo works, most can be totally cured, common in sub saharan africa bc epstein bar virus epidemic, kids and young adults