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Patient icterus

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					                   Patient 1
CC: 5YOWFpresents with fever, marked
weakness, pallor, bone pain, and bleeding from
her nose
HPI: progressively increasing fatigability and
infections over the past few months
PE: marked pallor; epistaxis; ecchymotic patches
over skin; sternal tenderness; slight
hepatosplenomegaly with nontender
lymphadenopathy; no signs of meningitis; normal
funduscopic exam.
Gross Pathology
 Neoplastic infiltration of lymph nodes,
 spleen, liver and bone marrow.
 Loss of normal bone marrow architecture.
Micro Pathology
 Myelophthisic bone marrow (distorted
 architecture secondary to a space-occupying
 lesion) with lymphoblastic infiltration
 Lymphoblasts with inconspicuous nucleoli,
 condensed chromatin, scant cytoplam
Labs
 Normocytic, normochromic anemia
 Absolute lymphocytosis with excess blasts
 (>30%)
 Negative monospot test for Epstein-Barr virus.
 Positive terminal deoxytransferase (TdT)(marker
 for immature T and B lymphocytes)
 Positive CD10 marker (CALLA-Common Acute
 Lymphoblastic Leukemia Antigen
Diagnosis and Discussion
 Diagnosis:
 Acute Lymphocytic Leukemia (ALL)
 Discussion:
 ALL is the most common pediatric
 neoplasm; it accounts for 80% of all
 childhood leukemias. It carries a good
 prognosis.
              Patient 2
CC: 25YOF presents with high grade fever,
menorrhagia, and marked weakness
HPI: recurrent infections over the last few
weeks
PE: Marked pallor; multiple purpuric
patches over skin; hepatosplenomegaly;
gingival hyperplasia; sternal tenderness;
normal funduscopic and neurologic exam
Gross Pathology
 Bone erosion due to marrow expansion
 Chloroma formation, mainly in skull
 Splenomegaly
Micro Pathology
 Auer Rods (basophilic cytoplasmic bodies)
 in myelocytes
 Peroxidase positive stains on bone marrow
 and gingival biopsy
 Myeloblasts with myelomonocytic
 differentiation replace normal marrow
Labs
 Normocytic, normochromic anemia.
 Thrombocytopenia
 Neutropenia.
 Prolonged PT/PTT
 Leukocytosis composed mainly of
 myeloblasts and promyelocytes
Diagnosis and Discussion
 Diagnosis:
 Acute Myelogenous Leukemia (AML)
 Discussion:
 Not as common as ALL. Increased risk
 associated with ionizing radiation, benzene
 exposure, Down’s syndrome, and cytotoxic
 chemotherapeutic agents
AML/ALL Common Symptoms
 Fever, pallor, weakness, bone pain,
 epistaxis, recurrent infections
 Thrombocytopenia; neutopenia
 Minor hepatosplenomegaly
    AML            vs.      ALL
Gingival lesions     Nontender
Auer Rods            lymphadenopathy
                     Scant cytoplasm
                     CD10 (CALLA)
                     positive
                     TdT positive
              Patient 3
11Month Old M of Indian descent
CC: Presents with marked pallor, failure to
thrive, and delayed developmental motor
milestones.
PE: Marked pallor, mild icterus; frontal
bossing and maxillary hypertrophy
“chipmunk face;” splenomegaly
Imaging
 XR, Skull (lateral): maxillary overgrowth
 and widening of diploic spaces with “hair
 on end” appearance of frontal bone, caused
 by vertical trabeculae
Gross Pathology
 Expansion of hematopoietic bone marrow
 Thinning of cortical bone
Micro Pathology
 Red marrow increased
 Yellow marrow decreased
 Marked erythoid hyperplasia in marrow
Labs
 Microcytic hypochromic anemia
 Decreased reticulocytosis
 Mildly increased unconjugated bilirubin
 Anisopoikilocytosis
 HbA-absent
 HbF-95%
Diagnosis and Discussion
 Diagnosis:
 Beta Thalassemia
 Discussion:
 Beta-Thalassemia results from decreased synthesis
 of beta-globulin chains due to errors in
 transcription, splicing or translation of mRNA.
 Alpha-Thalassemia results from decreased
 synthesis of alpha-globulin chain due to deletion
 of one or more of the four alpa genes normally
 present.
Treatment
 Blood transfusion, folic acid
 supplementation, iron chelation therapy,
 bone marrow transplantation
                 Patient 4
24YOWM
CC: Rapid enlargement of his abdomen,
producing a dragging sensation, along with a
painless lump in his neck for the past two months
HPI: Intermittent fever, drenching night sweats,
pruritus, and significant weight loss
PE: Pallor; unilateral nontender, rubbery, enlarged
cervical lymph nodes; splenomegaly; no
enlargement of tonsils
Labs
 Neutrophilic leukocytosis with lymphopenia
 Normocytic anemia
 Elevated ESR
 Elevated serum Cu and ferritin
 Negative Mantoux test
Imaging
 CXR: bilateral hilar lymphadenopathy
Gross Pathology
 Involved lymph nodes are rubbery
 Have “cut-potato” appearance of cut surface
Micro Pathology
 Lymph node biopsy shows large histiocyte
 cells with multilobed nuclei and
 eosinophilic nucleolus resembling owl’s
 eyes (Reed-Sternberg Cells)
 No bone marrow involvement.
Diagnosis and Discussion
 Diagnosis:
 Hodgkin’s Lymphoma
 Discussion:
 Four patterns seen on biopsy. Common
 symptoms are the fever, night sweats, and
 weight loss. The disease spreads to
 contiguous lymph nodes before moving into
 the blood.
              Patient 5
40YOWM
CC: Life insurance physical exam
HPI: No complaints except occasional
fatigue and increasing abdominal girth.
PE: Pallor of skin and mucus membranes;
markedly enlarged spleen; pain on palpation
of sternum; no lymphadenopathy
Gross Pathology
 Skull chloromas
 Enlarged congested spleen with areas of
 thrombosis and microinfarcts
 Hepatomegaly
Labs
 Markedly elevated WBC count (130,000)
 Immature granulocytes mixed with normal
 appearing ones
 Basophilia, eosinophilia, early thrombocytosis,
 late thrombocytopenia
 Low leukocyte alkaline phosphatase
 Elevated serum vitamin B12
 Chromosomal translocation t(9;22)/bcr-abl gene
 (Philadelphia Chromosome)
Micro Pathology
 Hepatic sinusoidal leukemic infiltrates
 Congestive splenomegaly with myeloid
 metaplasia
 Philadelphia chromosome in all myeloid
 progeny
Diagnosis and Discussion
 Diagnosis:
 Chronic Myelogenous Leukemia (CML)
 Discussion:
 Death usually results from accelerated
 transformation into acute leukemia (blast
 crisis) within 2-5 years.
               Patient 6
65YOWM
CC: Routine checkup
HPI: On directed history, he admits to a
weight loss of about 12lbs over the past 4
mos, together with episodes of epistaxis and
extreme fatigue
PE: Generalized nontender
lymphadenopathy; pallor; enlargement of
spleen and liver
Gross Pathology
 Lymph node enlargement
 Hepatosplenomegaly with tumor nodule
 formation
Micro Pathology
 Bone marrow biopsy reveals extensive
 infiltration, normal-looking lymphocytes
 and a few lymphoblasts
 Blood smear shows many lymphocytes with
 small, dark, round nucleus and scant
 cytoplasm
Labs
 Markedly elevated WBC (124,000)
 90% Lymphocytes
 No lymphoblasts
 Mild thrombocytopenia
 Cooms-positive hemolytic anemia
 Smudge cells
 B-cells express CD5 (normally a T-cell marker)
Diagnosis and Discussion
 Diagnosis:
 Chronic Lymphocytic Leukemia (CLL)
 Discussion:
 CLL is a malignant neoplastic disease of B
 Lymphocytes that express surface marker CD5.
 Characterized by slow progression of anemia,
 hemolytic anemia, recurrent infections, lymph
 node enlargement, and bleeding episodes.
              Patient 7
10YOBM
CC: Chronic nonhealing ulcer on lower leg
HPI: Recurrent episodes of abdominal and
chest pain along with diminution of vision.
PE: Fever; pallor; mild icterus; funduscopy
shows hypoxic spots with
neovascularization; nonhealing chronic
ulcer on left lower leg
Imaging
 CT/US of Abdomen:
 small, calcified spleen
Labs
 Decreased HCT
 Megaloblastic anemia
 Serum bilirubin moderately elevated
 Howell-Jolly bodies and Cabot rings
Diagnosis
 Sickle Cell Anemia
            Patient Next
10 Month old Female
CC: Mother claims the child is “a retard.”
She cannot see properly, and falls
repeatedly.
PE: No lacerations of fractures noted;
normal physical development for size and
weight; bruises in different stages of
healing; bilateral retinal hemorrhages
Imaging and Labs
 Imaging:
 XR: No new or old fractures
 Labs:
 Coagulation profile is normal.
Diagnosis and Discussion
 Diagnosis:
 Shaken Baby Syndrome---Abuse
 Discussion:
 Vigorous shaking can produce vitreous and retinal
 hemorrhages that may be the only verifiable sign
 of child abuse.
 We are required by law to report any suspicion of
 child abuse or neglect to state protection agencies
            Patient Last
64YOBM
CC: Bone pain, weight loss, easy
fatigability
HPI: Recurrent URIs and frequent nose
bleeds
PE: Pallor; bone tenderness in lower back
and ribs; petechiae on buccal mucosa; no
hepatosplenomegaly.
Labs
 Normocytic, normochromic anemia
 Neutropenia
 Rouleau formation
 Elevated Serum Ca++
 Normal alkaline phosphatase
 Increased ESR
 Gamma spike on serum protein electrophoresis
 UA: Bence Jones Proteinuria
Imaging and Gross Pathology
 Imaging:
 Punched out lytic bone lesions in vertebrae,
 long bones, and skull
 Gross Pathology:
 Multifocal replacement of normal bone
 tissue with tumor cells (plasmacytoma)
Micro Pathology
 Infiltration of bone marrow by normal-
 looking plasma cells.
 Amyloid deposits in kidney with renal
 tubular cast formation and interstitial
 fibrosis.
Diagnosis and Discussion
 Diagnosis:
 Multiple Myeloma
 Discussion:
 Primary malignancy of plasma cells with
 replacement of normal bone marrow; it is
 the most common primary bone cancer.

				
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