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Morning Report. Jieli Li 11/14/05. Chief Complaint. f/c, n/v, abdominal pain. HPI. 34 y/o AAM with hx of PSA presents with f/c, n/v and abdominal pain x 5 days.
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Morning Report Jieli Li 11/14/05
Chief Complaint • f/c, n/v, abdominal pain
HPI • 34 y/o AAM with hx of PSA presents with f/c, n/v and abdominal pain x 5 days. • Pt developed nausea and lack of appetite about 1 wk ago, followed by fevers and epigastric/RUQ pain. His mother who is a nurse told him to try Bye-lori 2(a herbal medication for H. pylori). Pt had no relief of symptoms • The morning of presentation pt had emesis x 1 in shower and a sharp pain in his right groin region where he had an old hernia years ago. He decided to come into clinic to seek medical help
HPI cont. • Pt also noticed a patchy rash on his abdomen and around both eyes and right upper back x 5 days. • + “tarry brown” stools for the last few days • + dark/concentrated appearing urine despite drinking plenty of water • Denies any cough, dysuria, grosshematuria, diarrhea/constipation, BRBPR.
PMH • PSA • etoh - quit in 1998, • ecstasy, LSD, cocaine - quit in 2000 • heroin - once (IVDA) • MJ and smoking – quit 2 months ago
History cont. • All: • Penicillin – hives, anaphylaxis • Meds: • Bye-lori 2 – homeopathic med for H. pylori • SH: • Pt works as an actor • His mother has been a nurse for 40 yrs • HIV, Hep C and other STD’s tested in 1994, neg per pt • No sexual activity since 02/2004 per pt • Denied homosexuality
Physical Exam • VS – T: 102.4, HR: 126, BP: 142/78, RR: 20 • Ht: 177.8 cm, Wt: 65.2 kg, BMI: 20.6 • Gen – thin but well-developed AAM in mild to moderate distress, + chills, + slight diaphoresis. AAO x 4 • Skin – slightly diaphoretic, + periorbital erythematous, non-blanching fine rash, also similar patchy rash on abdominal skin (RUQ and epigastric) and right upper back • HEENT – Mild jaundice, NC/AT, PERRLA, EOMI, no oral lesions • Neck – supple w/o LAD, no thyromegaly • Heart – tachycardic, regular rhythm, s1s2, no m/r/g • Lungs – cta bilaterally, no wheezes/crackles • Abd – scaphoid, hypoactive BS, + mild to moderate tenderness RUQ and epigastric region. No rebound, no guarding. No CVA tenderness. + hepatosplenomegaly. +small reducible hernia right groin. Non-tender. • Ext – no c/c/e • Neuro – CN II-XII grossly normal, non-focal
9.82 17.230.8 27.7 MCV 85.2, 32% neutrophils 20% lymphs, 43% monos, 4% basophils 130100 17 99 4.23 22 1.3 Ca , Mg , P Lactate 8.3 Stool ob positive PT 18.4,PTT 28.6, INR 1.6 Haptoglobin 5.83, direct and indirect Coombs neg Heterophile neg Alk phos 135, ALT 104, AST 192, total bili 3.7, direct bili 1.4 Amylase 52 U. tox - + marijuana UA – small bilirubin, small occult blood, 3 RBC, trace protein, urine urobilinogen > 8.0 Laboratory
Additional Labs • HIV NR • RPR NR • Malaria smear neg • Cryptococcus neg • CMV neg • Coccidioides neg • CSF cx’s (varicella, mycology, viral, bacterial, AFB) neg • Stool cx’s neg • VZV neg • Rocky Mountain Spotted Fever neg • Leptospira neg • Murine typhus neg • Ehrlichiosis neg • West nile virus neg
Ultrasound of Abdomen (10/03/05) • Liver and spleen are enlarged. They are homogeneous in echo texture without evidence of focal mass. No evidence of cholelithiasis or biliary dilatation. No definite pancreatic masses. • Kidneys are normal in size and in echotexture. • Main portal vein is patent. No evidence of ascites.
HIDA scan • No definite visualization of the gallbladder during approx 2 hours and 20 minutes of imaging after radiopharmaceutical injection. This is consistent with acute cholecystitis • There is no evidence of intrahepatic or common duct obstruction
CT abdomen/Pelvis (10/12/05) • Pericholecystic fluid distended gall bladder consistent with acute cholecystitis • Hepatosplenomegaly. Mild fatty change in the liver • Small amount of fluid within the pelvis • Again identified is distended urinary bladder
Hospital Course • Patient was initially admitted to MICU. Differential was broad given the constellation of symptoms. Multiple subspecialty services were consulted including ID, GI, Heme/onc, General surgery (for possible ischemic bowel and later for acalculous cholecystitis). Patient required multiple units of blood products to keep Hgb > 8 and plts > 50. • Pt had episodic melena and one episode of hematemesis as an inpatient, GI workup included a normal EGD, however pt refused colonoscopy. Pt was treated with 14 days of levofloxacin and flagyl for acute cholecystitis on HIDA and CT abdomen. Gen surgery plans to do cholecystectomy as outpatient.
Hospital Course • Pt developed throbbing headache lasting for 2-3 hrs. Spinal tap was done, which revealed possible aseptic meningitis. • Patient underwent induction chemo with cytarabine and idarubacin. Finished first cycle on 10/21/05, developed neutropenia with ANC down to 0. Pt was placed on modified neutropenic precautions (e.g, roams floor). However pt was non-compliant and refused to stay in room. He initially did well, but then spiked fever on 10/30. Was placed on multiple abx. Counts eventually rebounded and pt will be discharged home this week.
Introduction • A clonal disorder of the early myeloid cells. During normal hematopoiesis, myeloid blast cells differentiate into granulocytes, monocytes, erythrocytes, or megakaryocytes. • Malignant cells replace bone marrow, may infiltrate spleen, liver, lymph nodes and circulate in bloodstream • Usually less nodal involvement than ALL • 80% of adult leukemias vs. 20% for ALL; 20% of childhood leukemias vs. 80% for ALL • Can develop after transformation from myeloproliferative disorders, myelodysplastic disorders, and PNH • Overall 5-year survival is < 20%
Risk Factors • Down syndrome • Bloom syndrome • Fanconi’s anemia • Neurofibromatosis • Benzene exposure • Radiation • Alkylating agents • Type II topoisomerase inhibitors
Signs and Symptoms • Due to replacement of normal bone marrow cells by blasts • fatigue - due to anemia • fever • opportunistic infections - due to neutropenia • mucosal and cutaneous bleeding - due to thrombocytopenia • tissue infiltration (mainly with M4/M5) • sternal tenderness - due to bone marrow expansion • gingival hyperplasia • neurological symptoms - due to CNS infiltration
Diagnosis • Morphology • Myeloblasts have delicate nuclear chromatin, 2-4 prominent nucleoli, more voluminous cytoplasm than lymphoblasts in ALL, often with fine granules and Auer rods (abnormal crystallized granules, particularly in M3) • Monoblasts (M4, M5) have folded or lobulated nuclei, no Auer rods • Peripheral smear - 50% have WBC > 10,000, 20% > 100,000 due to circulating blasts and other immature myeloid cells • Bone marrow - hypercellular, 20% or more blasts (by definition) • Cytochemical tests • Immunophenotyping • CD99 – positive in 43% of AML • CD10 - usually negative vs. positive in ALL • Chromosomal analysis • 90% have chromosomal abnormalities • De novo cases often have balanced translocations
FAB Classification • M1 is AML without maturation • M2 is partially differentiated AML • > 50% of the cells being myeloblasts and promyelocytes and with myelocytes and metamyelocytes present • M3 is acute promyelocytic leukemia (APL) • > 30% promyelocytes • M4 is acute myelomonocytic leukemia (AMML) • resembles M2 except that approximately 25% of the cells are myeloblasts and promyelocytes and 25% are monoblasts and promonocytes/monocytes. • M5 is acute monocytic leukemia (AMoL) • M5a without maturation • M5b with partial maturation. • MB is erythroleukemia (EL) • > 50% or more nucleated bone marrow cells are erythroblasts and many having bizarre features.
Prognosticators • Cytogenetics • Low risk t(8;21),t(15;17), or inv(16) • Intermediate risk normal karyotype or t(19;11) • High risk inv (3), -5/del(5q), -7, or a more complex karyotype (3 or more aberrations) • Additional unfavorable prognosticators: • Age> 60 • Poor performancestatus • WBC count > 100,000/mm3 • Prior disease of the bone marrow (myelodysplasia or myeloproliferative disorder)
M3 Promyelocytic Variant • A favorable AML prognosticator • Associated with a translocation between chromosomes 15 and 17, involving the promyelocytic leukemia gene and retinoic acid receptor gene (PML-RAR) • Treatment: • All-trans retinoic acid (which will induce differentiation) • Daunorubicin • > 80% remission and > 70% cure rates
Promyelocytic Variant • Increased risk for developing DIC due to release of procoagulants from cytoplasmic granules
Chemotherapy • Indution therapy • Cytarabine + daunorubicin • Consolidation therapy • The same chemo regimen used for remission induction can be repeated for one or more cycles as consolidation treatment • Cure rate 10-30%
Other Treatment Options • Reserved for patients < 60 yrs old, with a histocompatible sibling and with poor prognostic cytogenetics • Autologous stem cell transplantation • Allogeneic bone marrow transplantation – Cure rate 45-65%