Download - Case report myelofibrosis
Resident on duty : dr. IrmawatiCoass on duty: Indra and AsisSupervisor : dr Soroy Lardo SpPD FINASIM
Ward ReportRSPAD Gatot Soebroto Jakarta Wednesday, 4th November 2015Suspect Myeloproliferative Disorders
Patient Recap New patients:
1. Mr. I – Anemia e.c. susp. Hypersplenism2. Mr. W – Hepatoma pro TACE.
PATIENT’S IDENTITY
Name : Mr. IAge : 74 years oldMR no. : 33.50.66Occupation : RetiredAddress : Komp.Palad RT03/06 CibinongReligion : IslamEthnicity: Jawa
ANAMNESIS
Chief Complain Pain on the upper left abdomen 3 days
prior to admission.
ANAMNESIS Present Illness:
Patient was admitted to the medical ward with complain of pain on the upper left abdomen 3 days prior to admission. The patients complains that the pain is continuous and describes the pain as a pinching pain. The severity of the pain is becoming worse with time. The pain does not radiate to other parts of the abdomen. There is no trigger to the pain, but the pain worsens if the patient lies on his left side. The pain reduces when the patient consumes pain-killer. Initially, the pain started 8 months prior to being admitted but the pain progressed to be unbearable since 3 days ago. The patient complains of not being able to sleep because of the pain and scores his pain on a scale of 6/10.
About a year ago, the patient suddenly felt fatigued and light-headed. The patient felt weak throughout his body and felt tired quickly. The patient came to the ER and was diagnosed with Anemia.
Nausea (-), Vomiting (-), fever (-), black stools (-), bloody urine (-), cough (-).
ANAMNESISHistory of Past Illness: •The patient has a history of heart disease which was diagnosed in 2009.•DM(-)•Hypertension (-)• Liver disease (-)
ANAMNESISHistory of Family Illness •Heart disease (-)•DM (-)• Liver disease (-)•Hypertension (-)
ANAMNESIS
History of medical treatment:The patient says that he has
undergone around seven times of blood transfusion but he doesn’t know the type of blood being given to him.
ANAMNESISSocial and Economic HistoryHistory of smoking (+)
1961-2009 (48 years) 2 packs a day
PHYSICAL EXAMINATION
General state : Moderately ill Consciousness : Compos mentis Blood Pressure : 130/80 mmHg Pulse : 96x/min, regular Respiratory rate : 18 x/min, kussmall (-), Temperature : 36.6 0 C Height : 165 cm Weight : 55 kg BMI : 20,2 m2/kg (normoweight)
PHYSICAL EXAMINATION
Head : NormocephalHair : Equal distribution, difficult to removeEyes : Pupil isokor, sclera icteric (-/-), pale
conjunctiva (+/+), Direct/Indirect pupillary reflex (+/+)
ENT : Normotia,deviated septum (-), hyperemia pharynx(-)
JVP : 5+0 cmH2O , Lymph nodes not palpableMouth : wet mouth mucousa,no oral lesions
PHYSICAL EXAMINATIONThorax :PulmoInspection : Symetric movement in
static and dynamic state, spider naevi (-), Gynaecomastia (-)
Palpation : Vocal tactile fremitus symetrical at both sides.
Percussion : Sonor on all lung fieldsAuscultation : Vesicular breath
sounds. Rh(-), Wh(-)
PHYSICAL EXAMINATION
Cor Inspection : Ictus cordis not seen Palpation : Ictus cordis palpable at ICS V
linea midclavivularis sinistra Percussion : All heart borders within
normal limit. Auscultation : Reguler S1 S2 heart
sounds. Murmur (-), gallop (-)
PHYSICAL EXAMINATIONAbdomen Inspection : Flat, scar (-), venectation (-),
jaundice (-), caput medusa (-) Auscultation : Bowel sound (+) Normal, Percusion : tympany in all regio, shifting dullnes
(-) Palpation : Soft, Tenderness (+) at Left
hypochondriac, hepatomegaly (-), splenomegaly (+) Schuffner 4, normal skin turgor .
Extremity : Warm acral, Oedema (-/-), CRT < 2 seconds, icteric (-)
Lymph node : Neck, Infraclavicular, supraclavicular, axillar and ingunal are not palpable.
LABORATORY EXAMINATION
Type of Exam
Result
Hemoglobin 7.8Hematocrite 24Erytrocyte 2.5Leucocyte 20890
Trombocyte 175000MCV 93MCH 31
MCHC 33Ureum 50
Creatinine 0.9RBG 93
Natrium 139Kalium 4.2
Chloride 103
02-11-2015
RADIOLOGIC EXAMINATIONUSGConclusion : Splenomegaly
SynopsisMale, 74 Y.O came with a chief complain of pain in
Upper left abdomen 3 days prior to admission. The pain is continuous and described as pinching pain. The pain does not radiate to other places. It is relieved with painkiller but worsens when the patient lays on his left side. The pain began at 8 months prior to admission but it progressed to be unbearable at 3 days ago. 12 months ago, the patient felt fatigue and he was diagnosed as anemia. Nausea (-), Vomiting (-), fever (-), black stools (-), bloody urine (-), cough (-).Patient has undergone blood transfusion 7 times in this one year.
In physical examination there are pale conjunctiva (+/+), Tenderness (+) at left hypochondriac region, Splenomegaly (+) Schuffner 4. In Laboratory examination, Hemoglobin 7.8, Hematocrite 24, Erytrocyte 2.5, Leucocyte 20890, MCV 93, MCH 31, MCHC 33.
The conclusion of the abdominal ultrasonography is Splenomegaly.
PROBLEM LIST1. Anaemia Normochromic Normocytic e.c
susp. Myeloproliferative disorder.
ASSESSMENTAnaemia Normochromic Normocytic e.c
Myeloproliferative disorder
Anamnesis :Pain at the left upper abdomen 3 days prior to admission, aggreviated when the patient lays on his left side. About a year ago, the patient suddenly felt fatigued and light-headed. Physical examination: Eye : Pale Conjunctiva (+/+)Abdomen : Tenderness (+) at left hypochondriac region,
Splenomegaly (+) Schuffner 4Laboratory examination:Hemoglobin 7.8 g/dlMCV 93 fLMCH 31 pgMCHC 33 g/dL
ASSESSMENT
Radiologic examination:USG conclusion: Splenomegaly
ASSESSMENTAnaemia Normocytic Normochromic e.c
Myeloproliferative disorderManagementDiagnostic Test: Complete Blood Count+ Diff count,Electrolyte Peripheral blood smear Bone Marrow Aspirate SGOT, SGPT, PT, APTT, Direct and Indirect
billirubin.
Treatment: Profenid Supp 1x1 Omeprazole 1x40 mg PO Consult on Haematology Department
Education: Need to consult about the condition every month or if there is a sign or symptom.
PROGNOSIS
Quo ad Vitam : dubiaQuo ad Functionam: dubia Quo ad Sanastionam : dubia
LITERATURE REVIEW
Physiologic of the hematologic cell lines
Definition of the myeloproliferative disorder
Myeloproliferative diseases are a heterogenous group of disorders characterized by cellular proliferation of 1 or more hematologic cell lines in the peripheral blood, distinct from acute leukemia.
In a small number of people, a myeloproliferative disorder progresses or transforms to leukemia, which is a cancerous (malignant) disorder.
Classification of the Hematologic malignancies
Major Myeloproliferative Disorders
Disorder Bone Marrow Characteristics Blood Characteristics
Polycythemia veraIncreased number of cells that produce the circulating blood cells
Increased number of red blood cellsOften, increased number of platelets and white blood cells
Myelofibrosis Excess fibrous tissue
Increased number of immature red and white blood cellsMisshapen red blood cellsDecreased overall number of red blood cells (anemia)The numbers of white blood cells and platelets often eventually decrease, but in some people they increase
ThrombocythemiaIncreased number of cells that produce platelets (megakaryocytes)
Increased number of platelets
Diagnostic Laboratory Complete blood count (CBC) and differential count with
microscopic examination of the peripheral smear Leukocyte alkaline phosphatase (LAP) score: To differentiate
chronic myelogenous leukemia from other causes of leukocytosis Polymerase chain reaction (PCR) assay or fluorescent in-situ
hybridization (FISH) run on peripheral blood: Can detect bcr-abl gene rearrangement; to differentiate chronic myelogenous leukemia from other myeloproliferative diseases
Red blood cell mass study: True versus spurious polycythemia Serum uric acid level PCR assay. To test for JAK2; available for suspected cases of
polycythemia vera, essential thrombocythemia, and myelofibrosis Biopsy
Management of Myeloproliferative DisordersNo curative drug treatment exists for
any of the MPDs. The goal of is prevention of thrombo-hemorrhagic complications. The goal of treatment for Myelofibrosis is amelioration of anemia, splenomegaly, and other symptoms. Low-dose aspirin is effective in Polycytemia Vera and Essential thrombocytopenia.
Thank You