Hematology and Oncology Flashcards by Jamaira Belmes

Progressive decline in Hgb that starts during 1st week of life that persists 6-8 weeks and usually lasts 8-12 weeks

Physiologic Anemia of Infancy

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The MC childhood malignancy

anorexia, irritability, lethargy

signs of marrow failure - anemia, bleeding, purpuric/petechial lesions, low grade fever

signs of infiltration - bone pain, lymphadenopathy - splenomegaly > hepatomegaly

Acute Lymphocytic Leukemia (ALL)

pancytopenia + hypercellular (blasts)

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The single most important prognostic factor in ALL

TREATMENT

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3 of the most important predictive factors in ALL

Speed of response to treatment
Age of the patient at the time of diagnosis
Initial leukocyte count

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Poor Prognostic Factors

< 2 yrs or >10 yrs
male
WBC > 100 000 u/L on presentation
(+) CNS leukemia
(+) mediastinal mass

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Sites of relapse in ALL

bone marrow

CNS – increased ICP and isolated nerve palsies

testes - painless swelling of one or both testes in 1-2 % of males

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Sites of metastases in ALL

liver
spleen
lymph nodes

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The MC hematologic disease of infancy and childhood

MC in 9-24 mos of age

MCC – poor dietary intake

Iron Deficiency Anemia (IDA)

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Most important sign of IDA

PALLOR - 7-8 mg/dL

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Reduction of the hemoglobin concentration or RBC volume below the range of values occurring in healthy persons

Anemia

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Macrocytic Anemia

MCV > 100

Folate Deficiency
Vitamin B12 Deficiency
Drug and alcohol induced anemia

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Microcytic Anemia

MCV < 80

Thalassemia
Anemia of Chronic Disease
IDA
Lead poisoning
Sideroblastic anemia

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Normocytic Anemia

MCV 80-100

Anemia of Chronic Disease
Uremia
Hypothyroidism
BM failure (aplastic anemia)

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Non hematologic effects of IDA

Pica
Pagophagia - ICE
Neurocognitive effects

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Diagnostic findings in IDA

low reticulocyte count
microcytic, hypochromic RBCs
high RDW
high TIBC
low serum iron and ferritin

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microcytic, hypochromic
low reticulocyte count
HIGH RDW

Iron of Deficiency Anemia

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microcytic, hypochromic
low reticulocyte count
NORMAL - DECREASED RDW

Thalassemia

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IDA vs Chronic Disease of Anemia

IDA - TIBC - HIGH

Anemia of Chronic Disease - TIBC - LOW

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Inherited microcytic hemolytic anemia d.t. abnormal hemoglobin synthesis

Result when 1 or more of globin gene mutates

Thalassemia

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Due to decrease in alpha-globin chain synthesis

Alpha Thalassemia

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Diagnosis of Alpha Thalassemia

DECREASED reticulocyte count (d.t. ineffective hematopoiesis)
microcytic hypochromic RBCs
normal RDW
TARFET CELLS
HEINS BODIES

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Treatment of Alpha Thalassemia

frequent transfusions
chelation therapy - Deferoxamine
Splenectomy

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Due to decrease in beta-globin chain synthesis or absence of beta chain of hemoglobin

Beta Thalassemia

Beta thalassemia major (Cooley anemia)
Beta thalassemia minor

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Treatment of Beta Thalassemia

aggressive transfusions
chelation therapy as needed
hydroxyurea
splenectomy

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Normochromic, normocytic RBCs | Increased MCHC

Spherocytosis

Results from decrease in RBC survival pallor jaundice d.t. increased indirect bilirubin increased LDH

HEMOLYTIC ANEMIA

Characteristic findings in Hemolytic Anemia

normocytic, normochromic RBCs | increased reticulocyte count

Etiologies of Hemolytic Anemia

Intrinsic RBC defects -- structural or metabolic abnormalities Autoimmune destruction Intravascular destruction

Hemolytic anemia d.t. intrinsic RBC defects

Spherocytosis | Sickle Cell Disease

Complications of Sickle Cell Disease

painful (vaso-occlusive) crisis splenic sequestration crisis -- auto-splenectomy -- increased susceptibility to encapsulated organisms aplastic crisis priapism stroke leg ulcers acute chest syndrome

Idiopathic Usually with VIRAL trigger MUCOSAL bleeding normal Hgb and WBC DECREASED platelet

Idiopathic Thrombocytopenic Purpura

Treatment of ITP

Supportive Steroids IVIG

X linked recessive deficiencies of factor VIII and factor IX

HEMOPHILIA Hemophilia A (factor VIII) - MC Hemophilia B or Christmas Disease (factor IX)

The MC hereditary BLEEDING disorder

von Willebrand disease

The MC and most serious congenital and coagulation factor deficiencies

Hemophilia A

The MC hereditary HYPERCOAGULABLE disorder

Factor V Leiden

Hallmark of Hemophilia

PROLONGED BLEEDING

Earliest joint hemorrhages in children

ANKLES

Diagnosis of von Willebrand disease

``` DECREASED vWF and factor VIII levels PROLONGED bleeding time ABNORMAL platelet adhesion INCREASED PTT Ristocetin cofactor assay - measures vWF antigen levels and activity ```

Treatment of von Willebrand disease

MILD - DESMOPRESSIN - causes release of vWF from endothelial stores SEVERE - Factor VIII (contain high vWF Ag)

Platelet dysfunction

bruising | mucosal bleeding -- epistaxis, menorrhagia

Coagulation disorder

joint bleeding | muscle bleeding

Normal PT, bleeding time and platelet count PROLONGED PTT

Hemophilia A and B

normal PT normal or decreased platelet count PROLONGED PTT and BLEEDING TIME

von Willebrand disease

normal PT and PTT PROLONGED BLEEDING TIME DECREASED platelet count

ITP

normal bleeding time and platelet count normal or prolonged PTT PROLONGED PT

Vitamin K deficiency

PROLONGED PT, PTT and BLEEDING TIME DECREASED PLATELET COUNT

DIC

Renal tumor of embryonal origin 2nd MC malignant abdominal tumor in childhood

WILMS TUMOR Associated with: Neurofibromatosis Beckwith Wiedemann syndrome (hemihypertrophy, visceromegaly, macroglossia) WAGR syndrome (Wilms tumor, Aniridia, Genitourinary abnormalities, Mental retardation)

PAINLESS abdominal enlargement with flank mass that does NOT cross the midline Hematuria, Hypertension

WILMS TUMOR kidneys - origin/location

Embryonal tumor of neural crest cell origin 3rd MC pediatric cancer

NEUROBLASTOMA Associated with: N-myc oncogene - tuberous sclerosis Neurofibromatosis - pheochromocytoma Hirschprung disease

Abdominal mass that CROSSES the midline Horner syndrome HPN cord compression (from a paraspinal tumor)

NEUROBLASTOMA anywhere along the neuroaxis (adrenals- MC) - origin/location

Site of metastasis in Wilms tumor

LUNGS

Site of metastasis in Neuroblastoma

BONES

Diagnosis of Wilms tumor

Imaging | Biopsy

Diagnosis of Neuroblastoma

Imaging Biopsy (BMA) Urine VMA and HVA

Differential Diagnosis of Small Round Blue Cell Tumors

WARMERPN ``` Wilms tumor Acute leukemia Rhabdomyosarcoma Mesothelioma/Medulloblastoma Ewing Sarcoma Retinoblastoma Primitive Neuroectodermal tumor (PNET) Neuroblastoma ```

Typical PBS findings in APLASTIC ANEMIA

pancytopenia | HYPOCELLULAR

Pancytopenia

Fanconi anemia | Aplastic Anemia

Treatment of Aplastic Anemia

RBC and platelet transfusion Allogenic BM transplant (sibling) Granulocyte colony-stimulating factor or Granulocyte macrophage colony-stimulating factor

MC solid tumor

Brain tumors

MC solid tumor OUTSIDE CNS

Neuroblastoma

MC soft tissue tumor

Rhabdomyosarcoma

Malignancy with HIGHEST mortality

Brain (PNET)

2nd decade MALES spindle cell producing OSTEOID METAPHYSIS of long bones local pain and swelling often with history of injury SUNBURST PATTERN

OSTEOSARCOMA

2nd decade MALES undifferentiated small round cell of neural origin DIAPHYSIS of long bones local pain and swelling with fever ONION SKINNING/MOTH EATEN APPEARANCE

EWING SARCOMA

Minimal Hgb levels in physiologic anemia of prematurity

7-9 g/dL

RESPONSE TO IRON THERAPY

12-24 hrs - subjective improvements 26-48 hrs - initial bone marrow response 28-72 hrs - reticulocytosis 4-30 days - increasing Hgb levels 1-3 mos - repletion of stores

1st lab value to decline in IDA

serum FERRITIN

Definitive diagnosis of thalassemia and SCD

Hb Electrophoresis

Hereditary Spherocytosis Confirmatory Test

Osmotic Fragility Test

Curative for hereditary spherocytosis

Splenectomy

Mutation in Sickle Cell Disease

MISSENSE mutation

At the end of induction therapy, peripheral blast count or minimal residual disease must be

<0.01 %

WAGR syndrome

Wilms tumor Aniridia GU malformation Mental retardation

Beckwith-Wiedemann syndrome

``` Visceromegaly Macroglossia Omphalocele Hyperinsulinemic hypogycemia Wilms tumor, Hepatoblastoma ```

Denys-Drash syndrome

Nephropathy Renal Failure Male pseudohermaphrodism Wilms tumor

Malignancy that may present with raccoon eyes, subcutaneous tumor nodules, "dancing eyes, dancing feet"

Neuroblastoma

Treatment for neuroblastoma | Stage 4S

Observation