DIC Disseminated Intravascular Coagulopathy

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DIC (Disseminated Intravascular Coagulopathy )

• Cx- sepsis, metastatic cancer, obstetric
• complication, trauma, malignancy, acute
  pancreatitis, nephrotic syndrome
• Px-The coagulation system is activated leading to
  microthrombi and global consumption of coag
  factors and platelets

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DIC

• Clx- presents with bleeding and/or thrombosis
• Dx- ?coag factors,(?PT,?PTT) and
• ? platelets in appropriate clinical setting.
• Helmet-shaped cells and schistocytes
• Tx- is to relieve the initiating cause

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DIC
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Bleeding Disorders

• Bleeding associated with thrombocytopenia(few
  circulating platelets) is distinctive
• Petechiae
• Mucosal bleeding
• Deep tissue bleeding such as hemarthroses is more
  typical of hemophilia

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Bleeding Disorders

• Platelet Abnormalities (microhemorrhage)
• Sx- Mucous Membrane bleeding,petechiae,
  purpura, prolonged bleeding time
• Cx- ITP(antiplatelet ab and ?Megakaryocyte)
• TTP, drugs and DIC

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Bleeding Disorders

• Coagulation factor defects (macrohemorrhage)
• Sx- Hemarrthroses (bleeding into joints), easy
  bruising, prolonged PT and/or PTT
• Cx- Coagulopathies like
• hemophilia A(factor VIII deficiency) (A sounds
  like 8)
• hemophilia B (factor IX deficiency)
• Von willebrands antigen (its deficiency)
• the most common bleeding disorder

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PT vs PTT

• PT (Extrinsic) PTT (Intrinsic)
• Prothrombin time Partial thromboplastin
  time
• 
  (Not VII, nor XIII)

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(No Transcript)
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Platelets, Coagulation factors and vWF
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Hereditary Platelet Dysfunction

• Glanzmanns thrombasthenia (gpIIbIIIa)
• autosomal recessive
• deficiency of gpIIbIIIa
• failure to aggregate with ADP
• Bernard Soulier Syndrome (gpIb)
• autosomal recessive
• Deficiency of gpIb
• failure to adhere

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Hemorrhagic disorders

• Disorder Platelet count
  Bleeding Time PT PTT
• Qualitative platelet defect ---
  --- ---
• Vascular bleeding ---
  --- ---
• Thrombocytopenia
  --- ---
• Hemophilia A (VIII) --- ---
  ---
• (sounds like 8)
• Hemophilia B (IX) --- ---
  ---
• aka Xmas factor
• Von Willebrands disease
  --- ---
• Disseminated Intra.. C
  

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Sorry, tis all about the USMLE

• A 25year old presents to the ER with mental
  status changes, diffuse ecchymotic lesions,
  granular brown emesis, and occult blood in the
  stool. The patient is also oozing blood from the
  site of IV access. WBC is 74,000/mm3. Blood smear
  shows a large number of immature myeloid cells
  (promyelocytes) with Auer rods. A coagulation
  profile is sent.
• Which of the following coagulation profiles
  would be expected?
• A) PTT 34S, PT 13S, PLATELETS 25,000
• B) PTT 34S, PT 14S, PLATELETS 235,000
• C) PTT 34S, PT 24S, PLATELETS 252,000
• D) PTT 55S, PT 13S, PLATELETS 255,000
• E) PTT 57S, PT 25S, PLATELETS 25,000

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Lymphomas

• Any neoplasms of the lymphoid tissue
• Can be suggested from clinical features but
  histological examination is necessary diagnosis
• So.. To Hodgkins or not to Hogkins

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Hodgkins

• Reed-Steinberg Cells CD30 and CD15
• Localized, single group of nodes extranodal
  rare, contiguous spread
• Sx- Low-grade fever, night sweats, weight
  loss
• Mediastinal Lymphadenopathy
• 50 of cases associated with EBV
• Bimodal- Young and old affected
• gtMen except the nodular sclerosis type
• Good prognosis when, lymphocytes RS

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Hodgkins
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Classical Hodgkins Lymphoma

• All have RS cells and its variants
• All are CD30 and CD15 positive
• All have few neoplastic cells in a background of
  non-neoplastic cells
• They are Nodular Sclerosing(NS), Mixed
  Cellularity(MC), Lymphocyte rich (LP?),
  lymphocyte depleted (LD)

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Classical Hodgkins Lymphoma

• Type RS Lymphocytes
  Prognosis Comments
• NS (65-75)
  Excellent lacunar
  cells
• collagen banding
• womengtmen
• Young adults
• MC (25)
  Intermediate Lots of RS cells
• LP (6) Excellent
  lt35YO male
• LD (rare) Higher
  Poor Older males with
• disseminated disease

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Non-Hodgkins

• Associated with HIV and Immunosuppresion
• Multiple, peripheral nodes, extranodal
  involvement
• Noncontiguous spread
• Majority involve B cells
• No hypergammaglobulinemia
• Fewer constitutional signs/symptoms
• Peak incidence (20-40YO)
• Not the old nor the young

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Non-Hodgkins lymphomas

• B-cell lymphoma
• I Precursor (immature) B-cell
  leukemia/lymphoma
• II Peripheral (mature) B-cell lymphomas
• 1. Small lymphocytic lymphoma
• 2. Prolymphocytic leukemia
• 3. Marginal zone lymphoma
• 4. Lymphoplasmacytic lymphoma
• 5. Hairy cell leukemia
• 6. Mantle cell lymphoma
• 7. Follicular lymphoma
• 8. Diffuse large B - cell lymphoma
• 9. Burkitt lymphoma
• 10. Plasma cell myeloma/plasmacytoma
• T-cell lymphoma
• I Precursor (immature) lymphoblastic T-cell
  lymphoma/leukemia
• II Peripheral (mature) T/NK lymphoma
• 1. T-cell prolymphocytic leukemia
• 2. T-cell granular lymphocytic leukemia
• 3. Aggressive NK-cell leukemia

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Non-Hodgkins Lymphoma

• Type Whom Cell
  Type Genetics Comments
• Small Lymphocytic Adults B cells clinically
  like
• Lymphoma CLL low grade
• Follicular lymphoma Adults B cells
  t(1418) Most common
• bcl-2 difficult to cure
• indolent course
• bcl-2 in apopt
• Burkitts lymphoma Children B cells
  t(814) Starry-sky
  assoc. EBV Jaw lesion
  in endemic form
• in Africa

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Non-Hodgkins
Burkitts Starry-sky
SLL
Follicular Cleaved cell
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Non-Hodgkins

• Type Whom
  Cell Type Comments
• Diffuse large older adults 80 B cells
  Aggressive
• Cell 20 in children 20 T
  cells up to 50
• (mature) curable
• Lymphoblastic Children most T cells
  Most common in
• Lymphoma (immature) children,
• presents with ALL
• and mediastinal
  mass
• Very aggressive

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Ann Arbor Staging

• I Single lymph node or single extralymph organ
• II 2 or more sites on same side of diaphragm
• III 2 or moresites on both sides of diaphragm
• IV Disseminated
• A W/out constitutional symptoms
• B with constitutional symptoms fever, night
  sweats, weight loss

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Leukemias

• Increasing number of circulating leukocytes in
  blood
• Bone marrow infiltrates of leukemic cells
• Marrow failure can cause anemia, infections and
  hemorrhage
• Leukemic infiltrates in liver, spleen, lymph nodes

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• Leukemia
• increased leukocytes
• Full bone marrow

Acute Leukemia Blasts predomainate Children or
Elderly Short and drastic
Chronic Leukemias More mature cells Midlife Age
Range Longer, less devastating
ALL Lymphoblasts (Pre-B or Pre-T)
AML Myeloblasts
CLL Lymphocytes Non-antibody- Poducing B cells
CML Myeloid stem cells Blast Crises
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ALL
AML

• Auer rods
• Myeloperoxidase

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CHRONIC LEUKEMIAS

• CLL CML
• Older adults
  Philadelphia Xmosome
• lymphadenopathy t(922), bcr-abl
• Hepatosplenomegaly Myeloid stem cell
  proliferation
• Indolent course ?Neutrophils and metamyelo
• ?smudge cells may accelerate to AML
• Tissue phase of SLL (blast crises)

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CHROMOSAL TRANSLOCATIONS

• DISORDER TRANSLOCATION
• CML (bcr-abl) t(922) aka?
• Burkitts (c-myc) t(814)
• Follicular (bcl-2) t(1418)
• Mantle cell t(1114)
• Ewings Sarcoma t(1122)
• M3 type AML t(1517)
• (responsive to ATRA)

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Lymphomas and Leukemias
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Multiple Myeloma

• Plasma Cell
• Terminally differentiated B lymphocyte
• Committed to antibody production
• Normally reside in the bone marrow
• Account for 1 of nucleated cells in bone
• marrow

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Multiple Myeloma

• Monoclonal Plasma cells (?Fried egg?) that arises
  in the bone marrow
• Lots of IgG (55) and IgA (25)
• Common tumor in bone in adults
• Hypercalcemia cos bone is destroyed
• Renal insufficiency
• Susceptible to infections and anemia

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Multiple Myeloma

• Monoclonal immunoglobulin
• spike (M protein) on
• protein electrophoresis
• Ig light chains in urine
• Bence Jones Protein

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Multiple Myeloma- Fried egg?
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Multiple Myeloma

• See punched out lytic bones on xray
• Bone pain,
• hypercalcemia

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Multiple Myeloma
Blood smear shows RBCs stacked like poker
chips (Rouleaux) formation
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Not again!

• 2) A 12-year-old girl with a history of multiple
  infectious diseases, fever, malaise, and fatigue
  is brought to her pediatrician. The physician
  draws a blood sample which reveals profound
  anemia and leukocytosis. Peripheral smear reveals
  that the white cells are largely leukemic blasts.
  The diagnosis of acute myelogenous leukemia is
  made. Which of the following may aid in the
  diagnosis of this condition?
• Aschoff bodies
• Auer bodies
• Bence-jones protein
• HLA B-27
• Ghon Complex

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Achalasia

• Failure of relaxation of lower esophageal
  sphincter
• Causes progressive dysphagia
• Barium swallow shows dilated esophagus with
  distal stenosis
• Associated with increased risk of esophageal
  carcinoma
• Secondary achalasia may arise from Chagas
• Bird beak on barium swallow

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Achalasia
Bird beak on Barium Swallow
Sorry just love this picture