Hemolyticuremic Syndrome

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ID/CC HPI PE Labs

Imaging Gross Pathology Micro Pathology

Treatment Discussion

An 8-year-old white male presents with an erythematous skin rash over the buttocks and legs coupled with joint pains and hematuria.

Three days before he had complained of cough, coryza, low-grade fever, and sore throat. He has a history of allergy to dust and pollen.

VS: hypertension. PE: palpable purpuric skin lesions over buttocks and legs; painful restriction of knee and ankle joint movement with swelling.

CBC: normal platelet count; normal coagulation tests. Increased ESR; increased BUN and serum creatinine. UA: RBCs and RBC casts on urinary sediment. Positive stool guaiac test (due to occult blood).

Necrotizing vasculitis of kidneys and lungs.

Renal biopsy shows focal and segmental glomerulonephritis with crescents (mesangioproliferative); mesangial IgA deposits on immunofluorescence.

Supportive; steroids; high-dose immunoglobulin therapy experimental.

An idiopathic disorder also known as anaphylactoid or vascular purpura; common vasculitis in children.

HENOCH-SCHÖNLEIN PURPURA

ID/CC

HPI PE Labs imaging Gross Pathology

Micro Pathology

Treatment Discussion

A 24-year-old white male complains of rapid enlargement of his abdomen, producing a dragging sensation, along with a painless lump in his neck for the past two months.

The patient also complains of intermittent fever, drenching night sweats, pruritus, and significant weight loss.

Pallor; unilateral nontender, rubbery, enlarged cervical lymph nodes; splenomegaly; no enlargement of tonsils.

CBC/PBS: neutrophilic leukocytosis with lymphopenia; normocytic anemia. Elevated ESR; elevated scrum copper and ferritin; negative Mantoux test.

CXR: bilateral hilar lymphadenopathy.

Involved lymph nodes are rubbery and have "cut-potato" appearance of cut surface.

Lymph node biopsy shows large histiocyte cells with multilobed nuclei and eosinophilic nucleolus resembling owl's eyes (= RE£D-STERNBERG CELLS); no bone marrow involvement on bone marrow biopsy.

Radiotherapy and chemotherapy.

Four patterns of Hodgkin's disease are seen on lymph node biopsy: lymphocytic predominance 10%-20%; nodular sclerosis 40%-60% (seen frequently in young women); mixed cellularity 20%-40%; and lymphocyte depleted 10%. Prognosis worsens in this order. Ann Arbor staging: I-IV with subclassification A (no constitutional symptoms) and B (weight loss, fever, night sweats).

HODGKIN'S LYMPHOMA

ID/CC HPI

PE Labs imaging Gross Pathology

Micro Pathology

Treatment Discussion

A 3-year-old white female is brought to the emergency room with a skin rash and severe epistaxis.

The patient had a URI consisting of a severe cough and a runny nose five days before the onset of her symptoms. She has a history of prolonged bleeding following minimal trauma; her father has been diagnosed with a bleeding disorder.

Mucosal petechiae; epistaxis; hemorrhagic bullae in buccal mucosa.

CBC: mild anemia; low platelet count (10,000); RBCs and "WBCs normal. Prolonged bleeding time; normal PTT; normal PT.

Purpura (due to extravasation of blood from intravascular space into skin); pin-sized hemorrhages (= PETECHIAE); ecchymosis (larger than petechiae but smaller than purpura).

Normal bone marrow aspirate with increased number of megakaryocytes.

Prednisone; splenectomy; danazol.

An autoimmune disease with formation of IgG anti-platelet antibodies and subsequent platelet destruction in the spleen.

IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)

ID/CC A 64-year-old black male suffers from bone pain, weight loss, and easy fatigability.

HP| He also complains of recurrent URIs.

PE Pallor; bone tenderness; petechiae on buccal mucosa; no hepatosplenomegaly.

Labs CBC/PBS: normocytic, normochromic anemia;

neutropenia; rouleau formation (RBCs adhering together like stack of poker chips). Elevated serum calcium; normal alkaline phosphatase; markedly increased ESR; gamma spike on serum protein electrophoresis (monoclonal gammopathy). UA; Bence Jones proteinuria (due to IgG light chains).

XR-Plain: punched-out, lytic bone lesions in vertebrae, long bones, and skull (axial skeleton).

Multifocal replacement of normal bone tissue with tumor cells (plasmacytoma); pelvis, skull, and spine most affected.

Infiltration of bone marrow by normal-looking plasma cells (abundant cytoplasm, eccentric nuclei) in aggregates; amyloid deposits in kidney with renal tubular cast formation and interstitial fibrosis (can cause renal insufficiency); bone erosion and destruction of cortical bone.

Treatment Various combinations of alkylating agents.

Discussion A primary malignancy of plasma cells with replacement of normal bone marrow; the most common primary bone cancer. p.227

Imaging Gross Pathology Micro Pathology

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