Gen Surg: Heme-Onc

Iron Deficiency Anemia

Microcytic anemia with decreased reticulocyte count and increased RDW due to a deficiency of iron

-MCC is chronic blood loss (US) or diet (worldwide)

-Sx: fatigue, SOB, HA, pagophagia, pica

-PE: koilonychia, angular cheilitis, tachycardia, pallor

-CBC shows microcytic hypochromic anemia with increased RDW, increased TIBC, and decreased ferritin

Ferrous sulfate

Treatment of choice for iron deficiency anemia?

Alpha Thalassemia

Microcytic anemia that is MC in SE Asians, associated with Hgb Barts and hydrops fetalis if —/—

-Sx: moderate anemia, jaundice, hepatosplenomegaly, gallstones, occasional need for transfusion during illness

-Dx: Hb Barts on newborn screen

-Tx: supplement folic acid, stem cell transplant is curative

Beta Thalassemia

Microcytic anemia that is MC in Mediterranean individuals, can require lifelong RBC transfusions

-Sx: hepatosplenomegaly, hemolytic anemia, maxillary prominence, frontal bossing

-Dx: can see HbF and HbA2 in Major

-Tx: chronic transfusion therapy

B12 Deficiency

Macrocytic anemia due to decreased absorption, leading to abnormal DNA synthesis

-Sx: fatigue, exercise intolerance, pallor, glossitis, diarrhea, malabsorption, symmetrical paresthesias, spinal cord demyelination and degeneration

-Dx: CBC shows megaloblastic anemia with hypersegmented neutrophils, macro-ovalocytes. Elevated homocysteine and MMA

-Tx: IM B12

Folate Deficiency

Macrocytic anemia due to inadequate intake (alcoholics), leading to abnormal DNA synthesis

-Sx: fatigue, exercise intolerance, pallor, chlorosis, glossitis, ulcers, diarrhea

-Dx: CBC shows megaloblastic anemia with hypersegmented neutrophils and macro-ovalocytes, low reticulocyte count, elevated homocysteine, normal MMA

-Tx: PO folic acid

Anemia of Chronic Disease

Anemia due to decreased RBC production in the setting of chronic disease, MC due to chronic inflammatory conditions or malignancy

-Patho: increased hepcidin and ferritin, which blocks release of iron and sequesters iron into storage

-Dx: CBC shows mild normocytic normochromic anemia, decreased reticulocytes, normal/increased ferritin, decreased TIBC and iron

-Tx: tx underlying cause

Sickle Cell Disease

Group of inherited hemoglobinopathies secondary to the production of abnormal hemoglobin, complicated with an associated hemolytic anemia and vaso-occlusion

-Manifestations: pain crisis, fever, splenic sequestration, acute chest syndrome, aplastic crisis, stroke

-Dx: peripheral smear shows sickled erythrocytes, target cells, and Howell-jolly bodies that indicates functional asplenia

-Tx: manage symptoms

S. pneumoniae

In a patient with Sickle Cell Disease, what is the encapsulated organism that is most likely to cause sepsis?

Splenic Sequestration

Intrasplenic trapping of RBCs and platelets, leading to a decrease in Hgb with an acute enlargement of spleen that can lead to life-threatening anemia. Also associated with acute illness.

-Tx: follow Hgb and spleen size closely, give fluids

Acute Chest Syndrome

Fever, respiratory sx, and a new pulmonary infiltrate on CXR seen in Sickle Cell Disease patients

-Tx: Ampicillin + Ceftriaxone + Azithromycin

Aplastic Crisis

Marked anemia with reticulocytopenia, frequently secondary to parvovirus infection that causes a maturation arrest of RBC production in bone marrow for 1-2 weeks

-Sx: increased fatigue, pallor, fever

-Tx: PRBC transfusion if sx

G6PD Deficiency

X-linked recessive enzymatic disorder of RBCs that may cause episodic hemolytic anemia, MC in AA males

-Sx: neonatal jaundice, dark urine, pallor, tachycardia, nausea, abdominal pain, fever, irritability after exposure to exacerbating factor

-Dx: Smear shows schistocytes and Heinz bodies

-Tx: Treat symptoms, avoid offending food and drugs

Hereditary Spherocytosis

Autosomal dominant hereditary intrinsic hemolytic anemia, which is due to a deficiency in RBC membrane and cytoskeleton that causes sphere-shaped RBCs

-Sx: anemia, jaundice, splenomegaly, pigmented gallstones

-Dx: peripheral smear shows hyperchromic microcytosis that is 80% spherocytes, increased MCHC, + osmotic fragility, -Coombs test

-Tx: folic acid + splenectomy

AIHA

Acquired hemolytic anemia due to autoantibody production against RBCs

-Sx: pallor, fatigue, weakness, dyspnea, hemoglobinuria, jaundice, splenomegaly

-Dx: CBC and smear shows microspherocytosis, polychromasia, + Coombs test

-Tx: steroids

Paroxysmal Nocturnal Hemoglobinuria

Rare, acquired stem cell mutation where RBCs become deficient in GPI anchor surface proteins (deficiency in CD55 and CD59)

-Sx: hemoglobinuria + pancytopenia + unexplained thrombosis in atypical veins

-PE: dark, cola-colored urine during early morning or at night

-Tx: eculizumab

Hemophilia

X-linked recessive disorder occurring almost exclusively in males

-Sx: hemarthrosis (MC in ankles), excessive hemorrhage due to trauma and surgery or incisional bleeding, epistaxis, bruising

-Dx: prolonged aPTT

VIII

What factor is affected in hemophilia A?

IX

What factor is affected in hemophilia B (Christmas disease)?

XI

What factor is affected in hemophilia C (MC in Ashkenazi Jews)?

Von Willebrand Disease

Autosomal dominant disorder associated with ineffective platelet adhesion due to deficient or defective vWF, MC hereditary bleeding disorder

-Sx: epistaxis, bleeding gums, petechiae, purpura, bruising, menorrhagia, prolonged bleeding time after minor cuts

-Dx: prolonged PTT and bleeding time

-Tx: DDVAP`

ITP

Autoimmune-antibody reaction vs platelets with splenic platelet destruction, leading to consumptive thrombocytopenia. Seen most often in young children after an acute viral infection

-Sx: petechiae, bruising, purpura, bullae, bleeding of gums, and menorrhagia

-Dx: isolated thrombocytopenia

-Tx: self-limited in kids, IVIG and steroids in adults

TTP

Auto-antibodies vs ADAMTS13, leading to consumptive thrombocytopenia and hemolytic anemia

-Sx: fever, anemia, jaundice, thrombocytopenia, renal failure/uremia, and neuro sx

-Dx: thrombocytopenia, hemolytic anemia, normal coags

-Tx: plasmapheresis and steroids

HUS

Exotoxins damage the vascular endothelium, activating platelets and causing a consumptive thrombocytopenia and hemolytic anemia. Primarily seen in children with diarrhea prodrome

-Sx: renal failure/uremia, anemia, thrombocytopenia, jaundice

-Dx: elevated BUN/Cr, thrombocytopenia, hemolytic anemia, normal coags

-Tx: observation in most children, plasmapheresis if needed

Antibiotics

What should be avoided in someone with suspected HUS?

DIC

Pathologic clotting cascade activation, leading to widespread platelet consumption and diffuse bleeding. MC in young or elderly

-Sx: diffuse bleeding at venipuncture sites, in mouth/nose, extensive bruising, renal failure, gangrene, acutely ill

-Dx: hemolytic anemia, decreased fibrinogen, increased D-dimer/Pt/PTT, and severe thrombocytopenia

-Tx: reverse underlying cause

HIT

Acquired thrombocytopenia within the first 5-10 days of initiation of heparin

-Sx: bleeding, venous thrombosis, gangrene, organ infarction, skin necrosis

-Dx: thrombocytopenia, thrombosis, 14-C-serotonin release assay

-Tx: immediate DC of all heparin + start non-heparin anticoagulants

Hodgkin Lymphoma

Germinal B-cell malignancy originating in the lymphatic system. Typically presents in ages 15-35 or > 60

-RF: prior EBV infection

-Sx: painless LAD (neck MC, followed by axilla), mediastinal LAD or mass, hepatomegaly, splenomegaly, fever/chills, night sweats, weight loss, fever

-Dx: Reed-Sternburg cells on lymph node biopsy

-Tx: chemo + radiation

Non-Hodgkin Lymphoma

B-cell malignancy that causes painless LAD that spreads non-contiguously to extranodal sites like skin, GI tract, and brain

-Sx: painless LAD, bowel obstruction, fatigue, easy bruising, recurrent infections

-Dx: starry sky appearance on microscopy (Burkitt lymphoma)

-Tx: radiation/chemo if stage I, R-CHOP if high-grade

AML

MC acute form of leukemia in adults

-Sx: lethargy, malaise, anorexia, mucocutaneous bleeding, increased infections and fever, arthralgia

-Dx: >20% blasts on bone marrow biopsy, +auer rods

-Tx: chemo + bone marrow transplant

Allopurinol

Given the patient’s most likely diagnosis, what is the best pharmaceutical treatment?

-Patient is a 56 y/o female undergoing chemotherapy for her AML. She develops hyperkalemia, hypocalcemia, hyperuricemia, and renal failure. You suspect tumor lysis syndrome.

CML

A type of leukemia that is typically seen in older males and is characterized by a translocation between chromosomes 9 and 22, which is called the Philadelphia chromosome

-Sx: fatigue, night sweats, malaise, weight loss, fever, splenomegaly, pruritis after hot baths/showers

-Dx: Philadelphia chromosome, leukocytosis > 100,000

-Tx: PO chemo + tyrosine kinase inhibitor like Gleevec

ALL

Malignancy arising from immature lymphoid stem cells in the bone marrow, MC childhood malignancy

-Sx: fever, infections, bleeding, anemia, HA, stiff neck, visual changes, vomiting, arthralgias

-PE: hepatomegaly/splenomegaly, LAD

-Dx: >20% blasts, PAS +, ANC <1000

-Tx: chemo, stem cell transplant

CLL

MC leukemia in adults, defined as a mature B cell clonal malignancy

-Sx: fatigue, mucocutaneous bleeding, increased infections and fever, LAD, splenomegaly

-Dx: smudge cells

-Tx: chemo

50,000

What is the minimum platelet count for major procedures?