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USMLE 1 > Hematology and oncology- Phatology (2) > Flashcards

Hematology and oncology- Phatology (2) Flashcards

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USMLE® Step 1 flashcards
1
Q

Lead poisoning

- Clinical features

A

Microcytic anemia, GI and kidney disease.

Children—exposure to lead paint Ž mental deterioration.
Adults—environmental exposure (eg, batteries, ammunition) Ž headache, memory loss, demyelination

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2
Q

Acute intermittent porphyria

  • Etiology
  • Accumulate substrates
A 
Porphobilinogen deaminase (uroporphyrinogen I
synthase). AD

Porphobilinogen, ALA

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3
Q

Acute intermittent porphyria

  • Symptoms
  • Treatment
A 
(5 P’s):
ƒ Painful abdomen
ƒ Port wine–colored urine
ƒ Polyneuropathy
ƒ Psychological disturbances
ƒ Precipitated by drugs (eg, cytochrome P-450 inducers), alcohol, starvation

Treatment: hemin and glucose, which inhibit ALA synthase.

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4
Q

Porphyria cutanea tarda

  • Etiology
  • Accumulate substrates
  • Symptoms
  • Treatment
A

Uroporphyrinogen decarboxylase. AD

Uroporphyrin (teacolored urine)

Blistering cutaneous photosensitivity and hyperpigmentation.

Most common porphyria. Exacerbated with alcohol consumption. Associated with hepatitis C.

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5
Q

Iron poisoning

  • Mechanism
  • Symptoms
  • Treatment
A

Cell death due to peroxidation of membrane lipids.

Nausea, vomiting, gastric bleeding, lethargy, scarring leading to GI obstruction.

Chelation (eg, IV deferoxamine, oral deferasirox) and dialysis.

*high mortality rate with accidental ingestion by children.

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6
Q

Coagulation Tests

  • PT
  • INR
  • PTT
A

Tests function of common and extrinsic pathway (factors I, II, V, VII, and X).

Calculated from PT. 1 = normal, > 1 = prolonged.

Tests function of common and intrinsic pathway (all factors except VII and XIII)

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7
Q

Hemophilia A, B, or C

  • Test altered
  • Clinical manifestations
  • Treatment
A

Intrinsic pathway coagulation defect (High PTT).

Hemorrhage in hemophilia—hemarthroses easy bruising, bleeding after trauma or surgery.

Desmopressin + factor VIII concentrate (A); factor IX concentrate (B); factor XI concentrate (C).

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8
Q

Platelet disorders

  • Test altered
  • Clinical manifestations
  • Etiologies
A

Defects in platelet plug formation, increas bleeding time (BT).

Microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura.

Bernard-Soulier, Glanzmann thrombasthenia, HUS, PTT, Immune thrombocytopenia.

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9
Q

Bernard-Soulier syndrome

  • Etiology
  • Labs
A

Defect in platelet plug formation. Large platelets. GpIb Ž defect.

Abnormal ristocetin test that does not correct with mixing studies.

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10
Q

Glanzmann thrombasthenia

  • Etiology
  • Labs
A

Defect in platelet integrin αIIbβ3 (GpIIb/IIIa).

Labs: blood smear shows no platelet clumping.

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11
Q

HUS

  • Labs
  • Etiology
  • Treatment
A

thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure.

Typical HUS is seen in children, accompanied by diarrhea. (EHEC) (eg,O157:H7)

Same spectrum as TTP, with a similar clinical presentation and same initial treatment of plasmapheresis

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12
Q

Immune thrombocytopenia

  • Etiology
  • Labs
  • Treatment
A

Anti-GpIIb/IIIa antibodies, splenic macrophage consumption of platelet-antibody complex.

megakaryocytes on bone marrow biopsy.

Steroids, IVIG; rituximab or splenectomy for refractory ITP.

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13
Q

TTP

  • Etiology
  • Labs
A

Inhibition or deficiency of ADAMTS 13 (vWF metalloprotease). large vWF multimers, platelet adhesion, platelet aggregation and thrombosis.

schistocytes,  LDH, normal coagulation parameters.

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14
Q

TTP

  • Clinical manifestations
  • Treatment
A

(FAT RN): pentad of Fever, microangiopathic hemolytic Anemia, Thrombocytopenia, Renal failure, Neurologic symptoms.

Treatment: plasmapheresis, steroids.

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15
Q

Disseminated intravascular coagulation

- Etiologies

A

“STOP Making New Thrombi”

Sepsis (gram ⊝), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion

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16
Q

Disseminated intravascular coagulation

- Labs

A

schistocytes,  fibrin degradation products (d-dimers),  fibrinogen,  factors V and VIII.

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17
Q

Hereditary thrombosis syndromes leading to hypercoagulability

A

Antithrombin deficiency, Factor V Leiden, Protein C or S

deficiency, Prothrombin gene mutation

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18
Q

Antithrombin deficiency

- Etiologies

A

Inherited deficiency of antithrombin: diminishes the increase in PTT following heparin administration.

Can also be acquired: renal failure/nephrotic syndrome.

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19
Q

Factor V Leiden

  • Etiology
  • Epidemiology
A

Mutant factor V. Arg506Gln mutation. Resistant to degradation by activated protein C.

Most common cause of inherited hypercoagulability in Caucasians.

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20
Q

Protein C or S deficiency

  • Etiology
  • Complications
A

Decrease ability to inactivate factors Va and VIIIa.

risk of thrombotic skin necrosis with hemorrhage after administration of warfarin.

Together, protein C Cancels, and protein S Stops, coagulation.

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21
Q

Blood transfusion (Packed RBCs)

  • Effect
  • Clinical use
A

Increase Hb and O2 carrying capacity.

Acute blood loss, severe anemia

22
Q

Blood transfusion (Platelets)

  • Effect
  • Clinical use
A

Increase platelet count.

Stop significant bleeding (thrombocytopenia, qualitative platelet defects)

23
Q

Blood transfusion (Fresh frozen plasma (FFP)/prothrombin complex concentrate(PCC))

  • Effect
  • Clinical use
A

FFP contains all coagulation factors and plasma proteins; PCC contains factors II, VII, IX, and X, as well as protein C and S.

DIC, cirrhosis, immediate anticoagulation reversal

24
Q

Blood transfusion (Cryoprecipitate)

  • Effect
  • Clinical use
A

Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin

Coagulation factor deficiencies involving fibrinogen and factor VIII

25
Q

Hodgkin lymphoma

  • Spread
  • Characteristic cells
A

Localized, single group of nodes; contiguous
spread. Overall prognosis better than that of non-Hodgkin

Reed-Sternberg cells. 2 owl eyes × 15 = 30. RS cells are CD15+ and CD30+ B-cell origin.

26
Q

Hodgkin lymphoma

  • Occurs in
  • Associations
A

young adulthood and > 55 years; more common in men except for nodular sclerosing type.

Associated with EBV.

27
Q

Hodgkin lymphoma

- Types

A
  • Nodular sclerosis: Most common
  • Lymphocyte rich: Best prognosis
  • Mixed cellularity: Eosinophilia, seen in immunocompromised patients
  • Lymphocyte depleted: Seen in immunocompromised patients
28
Q

Non-Hodgkin lymphoma

  • Spread
  • Cells affected
  • Ocurrs in
  • Associations
A

Multiple lymph nodes involved; extranodal involvement common; noncontiguous spread.

Majority involve B cells; a few are of T-cell lineage.

in children and adults.

May be associated with HIV and autoimmune diseases.

29
Q

Burkitt lymphoma

  • Occurs in
  • Genetics
  • Cell morphology
  • Presentation
A

Adolescents or young adults.

t(8;14)—translocation of c-myc (8) and heavy-chain Ig (14)

“Starry sky” appearance, sheets of lymphocytes with interspersed “tingible body” macrophages. Associated with EBV.

Jaw lesion in endemic form in Africa; pelvis or abdomen in sporadic form.

30
Q

Diffuse large B-cell lymphoma

  • Occurs in
  • Genetics
A

Usually older adults, but 20% in children.

Alterations in Bcl-2, Bcl-6.

*Most common type of non-Hodgkin lymphoma in adults

31
Q

Follicular lymphoma

  • Occurs in
  • Genetics
  • Presentation
A

Adults

t(14;18)—translocation of heavy-chain Ig (14) and BCL-2 (18)

Presents with painless “waxing and waning” lymphadenopathy.

32
Q

Mantle cell lymphoma

  • Occurs in
  • Genetics
  • Presentation
A

Adult males

t(11;14)—translocation of cyclin D1 (11) and heavy-chain Ig (14), CD 5+

Very aggressive, patients typically present with late-stage disease.

33
Q

Marginal zone lymphoma

  • Occurs in
  • Genetics
  • Association
A

Adults

t(11;18)

Associated with chronic inflammation

34
Q

Primary central nervous system lymphoma

  • Occurs in
  • Association
A

Adults

Most commonly associated with HIV/AIDS; pathogenesis involves EBV infection.

*needs to be distinguished from toxoplasmosis via CSF analysis or other lab tests.

35
Q

Adult T-cell lymphoma

  • Occurs in
  • Association
  • Presentation
A

Adults

Caused by HTLV (associated with IV drug abuse)

present with cutaneous lesions; common in Japan, West Africa, and the Caribbean. Lytic bone lesions, hypercalcemia.

36
Q

Mycosis fungoides/ Sézary syndrome

  • Occurs in
  • Clinical Characteristics
A

Adults

Skin patches/ plaques (cutaneous T-cell lymphoma), characterized by atypical CD4+ cells with “cerebriform” nuclei and intraepidermal neoplastic cell aggregates
(Pautrier microabscess).

May progress to Sézary syndrome (T-cell leukemia).

37
Q

Multiple myeloma

- Clinical manifestations

A

CRAB:

HyperCalcemia
Renal involvement
Anemia
Bone lytic lesions/Back pain

38
Q

Multiple myeloma

- Associated with

A

ƒƒ susceptibility to infection
ƒƒ Primary amyloidosis (AL)
ƒƒ Punched-out lytic bone lesions on x-ray
ƒƒM spike on serum protein electrophoresis
ƒƒ Ig light chains in urine (Bence Jones protein)
ƒƒ Rouleaux formation B (RBCs stacked like poker chips in blood smear)
ƒƒ Bone marrow > 10% monoclonal plasma cells

39
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A

Bone marrow < 10% monoclonal plasma cells, asymptomatic, Develop myeloma at a rate of 1–2% per year. No CRAB findings.

40
Q

Waldenström macroglobulinemia

A

M spike = IgM Ž hyperviscosity syndrome (eg, blurred vision, Raynaud phenomenon); no CRAB findings.

41
Q

Myelodysplastic syndromes

A

Stem-cell disorders involving ineffective hematopoiesis Ž defects in cell maturation of nonlymphoid lineages.

Risk of transformation to AML.

*Pseudo–Pelger-Huet anomaly—neutrophils with bilobed (“duet”) nuclei. Typically seen after chemotherapy.

42
Q

Leukemias

A

Unregulated growth and differentiation of WBCs in bone marrow Ž marrow failure Ž anemia, infections (Lowmature WBCs), and hemorrhage (Low platelets)

43
Q

Acute lymphoblastic leukemia/lymphoma

  • Occurs in
  • Presentation
  • Markers
  • Prognosis
A

in children. T-cell ALL can present as mediastinal mass (presenting as SVC-like syndrome). Associated with Down syndrome.

Peripheral blood and bone marrow have increased lymphoblasts

TdT+ (marker of pre-T and pre-B cells), CD10+ (marker of pre-B cells).

Most responsive to therapy. May spread to CNS and testes. t(12;21) better prognosis.

44
Q

Chronic lymphocytic leukemia/small lymphocytic lymphoma

  • Occurs in
  • Markers
  • Presentation
A

Age > 60 years. Most common adult leukemia.

CD20+, CD23+, CD5+ B-cell neoplasm.

Often asymptomatic, progresses slowly; smudge cells. autoimmune hemolytic anemia.

45
Q

Richter transformation

A

CLL/SLL transformation into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL).

46
Q

Hairy cell leukemia

  • Occurs in
  • Clinical Features
  • Diagnosis
A

Adult males. Mature B-cell tumor. lymphadenopathy is uncommon.

Causes marrow fibrosis Ž dry tap on aspiration. Patients usually present with massive splenomegal and pancytopenia.

Stains TRAP (tartrate-resistant acid phosphatase) ⊕. TRAP stain largely replaced with flow cytometry.

47
Q

Acute myelogenous leukemia

  • Occurs in
  • Morphology
  • Risk factors
A

Median onset 65 years.

Auer rods; myeloperoxidase ⊕ cytoplasmic inclusions seen mostly in APL; circulating myeloblasts on peripheral smear.

Risk factors: prior exposure to alkylating chemotherapy, radiation, myeloproliferative disorders, Down syndrome.

*APL: t(15;17), responds to all-trans retinoic acid (vitamin A). DIC its a common presentation.

48
Q

Chronic myelogenous leukemia

  • Occurs in
  • Genetics
  • Presentation
  • Treatment
A

peak incidence 45–85 years, median age at diagnosis 64 years.

Defined by the Philadelphia chromosome (t[9;22], BCR-ABL) and myeloid stem cell proliferation.

Presents with dysregulated production of mature and maturing granulocytes and splenomegaly. May accelerate and transform to AML or ALL (“blast crisis”).

Responds to bcr-abl tyrosine kinase inhibitors (eg, imatinib, dasatinib)

49
Q

Chronic myeloproliferative disorders

A

polycythemia vera, essential thrombocythemia, myelofibrosis, and CML.

Associated with V617F JAK2 mutation.

*Pag 422

50
Q

Langerhans cell histiocytosis

  • Definition
  • Presentation
  • Markers and characteristic granules
A

Proliferative disorders of dendritic (Langerhans) cells.

Presents in a child as lytic bone lesions and skin rash or
as recurrent otitis media with a mass involving the mastoid bone.

Cells express S-100 (mesodermal origin) and CD1a. Birbeck granules (“tennis rackets” or rod shaped on EM) are characteristic.

51
Q

Tumor lysis syndrome

A
  • hyperkalemia
  • hyperphosphatemia, hypocalcemia due to Ca2+ sequestration by PO4
  • hyperuricemia Ž acute kidney injury

Prevention and treatment include aggressive hydration, allopurinol, rasburicase.

USMLE 1 (73 decks)

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